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Michael Welsh

Research Profile

Chemicals & Drugs

Disorders

Cystic Fibrosis

Anatomy

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Kishore Nannapaneni; Yehuda Ben-Shahar; Thomas L Casavant; Henry L Keen; Michael J Welsh; Todd E Scheetz
Computational identification of operon-like transcriptional loci in eukaryotes.
Justin S Feinstein; Robin L Follmer; Rene Hurlemann; Daniel Tranel; Michael J Welsh; Colin Buzza; William H Coryell; Nader S Dahdaleh; John A Wemmie
Fear and panic in humans with bilateral amygdala damage.
Leah R Reznikov; Richard J H Smith; David A Stoltz; Jeng-Haur Chen; Qian Dong; Jianyang Du; Michael S Hildebrand; Thomas O Moninger; Jung Min Park; Christoph O Randak; Yuzhou Zhang; Michael J Welsh
CFTR-deficient pigs display peripheral nervous system defects at birth.

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2013: Kishore Nannapaneni; Yehuda Ben-Shahar; Thomas L Casavant; Henry L Keen; Michael J Welsh; Todd E Scheetz
Computational identification of operon-like transcriptional loci in eukaryotes.
Computers in biology and medicine 2013;43(6):738-43.
2013: Justin S Feinstein; Robin L Follmer; Rene Hurlemann; Daniel Tranel; Michael J Welsh; Colin Buzza; William H Coryell; Nader S Dahdaleh; John A Wemmie
Fear and panic in humans with bilateral amygdala damage.
Nature neuroscience 2013;16(3):270-2.
2013: Leah R Reznikov; Richard J H Smith; David A Stoltz; Jeng-Haur Chen; Qian Dong; Jianyang Du; Michael S Hildebrand; Thomas O Moninger; Jung Min Park; Christoph O Randak; Yuzhou Zhang; Michael J Welsh
CFTR-deficient pigs display peripheral nervous system defects at birth.
Proceedings of the National Academy of Sciences of the United States of America 2013;110(8):3083-8.
2012: Christoph O Randak; Amanda R Ver Heul; Michael J Welsh
Demonstration of phosphoryl group transfer indicates that the ATP-binding cassette (ABC) transporter cystic fibrosis transmembrane conductance regulator (CFTR) exhibits adenylate kinase activity.
The Journal of biological chemistry 2012;287(43):36105-10.
2012: Aliye Uc; Marwa Abu-El-Haija; Maisam Abu-El-Haija; Radhamma Giriyappa; Michelle Griffin; Paula Ludwig; David K Meyerholz; Lynda S Ostedgaard; Alejandro Pezzulo; David A Stoltz; Peter J Taft; Xiao Xiao Tang; Michael J Welsh
Pancreatic and biliary secretion are both altered in cystic fibrosis pigs.
American journal of physiology. Gastrointestinal and liver physiology 2012;303(8):G961-8.
2012: Eugene H Chang; Sarah Ernst; Nicholas D Gansemer; Philip H Karp; Paul B McCray; David K Meyerholz; Thomas O Moninger; Alejandro A Pezzulo; Andrea E Potash; Leah R Reznikov; Jessica C Sieren; David A Stoltz; Tanner J Wallen; Michael J Welsh; Joseph Zabner
Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis.
The Laryngoscope 2012;122(9):1898-905.
2012: Shyam Ramachandran; Scott D Rose; Amy E Walz; Michael J Welsh; Yi Xing; Lynda S Ostedgaard; Mark A Behlke; John T Fisher; Ashley M Jacobi; Peng Jiang; Philip H Karp; Shaf Keshavjee; Kim A Lennox; Paul B McCray
A microRNA network regulates expression and biosynthesis of wild-type and DeltaF508 mutant cystic fibrosis transmembrane conductance regulator.
Proceedings of the National Academy of Sciences of the United States of America 2012;109(33):13362-7.
2012: Maisam Abu-El-Haija; Marwa Abu-El-Haija; Radhamma L Giriyappa; Michelle Griffin; Paul B McCray; David K Meyerholz; Shyam Ramachandran; David A Stoltz; Michael J Welsh; Aliye Uc
Pancreatic damage in fetal and newborn cystic fibrosis pigs involves the activation of inflammatory and remodeling pathways.
The American journal of pathology 2012;181(2):499-507.
2012: Alejandro A Pezzulo; Shyam Ramachandran; David A Stoltz; Xiao Xiao Tang; Martin van Eijk; Michael J Welsh; Christine L Wohlford-Lenane; Mahmoud H Abou Alaiwa; Botond Bánfi; Henk P Haagsman; Mark J Hoegger; Alexander R Horswill; Phillip H Karp; Paul B McCray; Thomas O Moninger; Joseph Zabner
Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung.
Nature 2012;487(7405):109-13.
2012: Vincent A Magnotta; Daniel R Thedens; Michael J Welsh; Nader S Dahdaleh; Brian J Dlouhy; Robin L Follmer; Hye-Young Heo; John A Wemmie
Detecting activity-evoked pH changes in human brain.
Proceedings of the National Academy of Sciences of the United States of America 2012;109(21):8270-3.
2012: Qian Dong; Lynda S Ostedgaard; Christopher Rogers; Daniel W Vermeer; Yuping Zhang; Michael J Welsh
Human-mouse cystic fibrosis transmembrane conductance regulator (CFTR) chimeras identify regions that partially rescue CFTR-ΔF508 processing and alter its gating defect.
Proceedings of the National Academy of Sciences of the United States of America 2012;109(3):917-22.
2012: Sinyoung Kang; Margaret P Price; Michael J Welsh; Christopher J Benson; Mamta Gautam; Huiyu Gong; Jun Ho Jang; Timothy J Brennan
Simultaneous disruption of mouse ASIC1a, ASIC2 and ASIC3 genes enhances cutaneous mechanosensitivity.
PloS one 2012;7(4):e35225.
2011: Omar A Itani; Philip H Karp; Shaf Keshavjee; Julia Klesney-Tait; Kalpaj Parekh; Jeng-Haur Chen; Sarah Ernst; Joseph Zabner; Michael J Welsh
Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance.
Proceedings of the National Academy of Sciences of the United States of America 2011;108(25):10260-5.
2011: V C Vralsted; Margaret P Price; J Du; Mikael Schnizler; A M Wunsch; A E Ziemann; Michael J Welsh; John A Wemmie
Expressing acid-sensing ion channel 3 in the brain alters acid-evoked currents and impairs fear conditioning.
Genes, brain, and behavior 2011;10(4):444-50.
2011: Daniel Lorentzen; Lakshmi Durairaj; Alejandro A Pezzulo; Yoko Nakano; Janice Launspach; David A Stoltz; Gideon Zamba; Paul B McCray; Joseph Zabner; Michael J Welsh; William M Nauseef; Botond Bánfi
Concentration of the antibacterial precursor thiocyanate in cystic fibrosis airway secretions.
Free radical biology & medicine 2011;50(9):1144-50.
2011: Lynda S Ostedgaard; David K Meyerholz; Jeng-Haur Chen; Alejandro A Pezzulo; Philip H Karp; Tatiana Rokhlina; Sarah E Ernst; Robert A Hanfland; Leah R Reznikov; Paula S Ludwig; Mark P Rogan; Greg J Davis; Cassie L Dohrn; Christine Wohlford-Lenane; Peter J Taft; Michael V Rector; Emma Hornick; Boulos S Nassar; Melissa Samuel; Yuping Zhang; Sandra S Richter; Aliye Uc; joel shilyansky; Randall S Prather; Paul B McCray; Joseph Zabner; Michael J Welsh; David A Stoltz
The ΔF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs.
Science translational medicine 2011;3(74):74ra24.
2011: Maisam Abu-El-Haija; John Butler; Paul B McCray; David K Meyerholz; Marek Sinkora; Michael J Welsh; Aliye Uc
An activated immune and inflammatory response targets the pancreas of newborn pigs with cystic fibrosis.
Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] 2011;11(5):506-15.
2011: Eugene H Chang; Rodrigo S Lacruz; Pablo Bringas; Timothy G Bromage; Michael J Welsh; Joseph Zabner; Michael L Paine
Enamel pathology resulting from loss of function in the cystic fibrosis transmembrane conductance regulator in a porcine animal model.
Cells, tissues, organs 2011;194(2-4):249-54.
2010: Jeng-Haur Chen; David A Stoltz; Philip H Karp; Sarah E Ernst; Alejandro A Pezzulo; Thomas O Moninger; Michael V Rector; Leah R Reznikov; Janice L Launspach; Kathryn Chaloner; Joseph Zabner; Michael J Welsh
Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia.
Cell 2010;143(6):911-23.
2010: David K Meyerholz; David A Stoltz; Alejandro A Pezzulo; Michael J Welsh
Pathology of gastrointestinal organs in a porcine model of cystic fibrosis.
The American journal of pathology 2010;176(3):1377-89.
2009: Yongjun Lu; Xiuying Ma; Rasna Sabharwal; Vladislav Snitsarev; Donald Morgan; Kamal Rahmouni; HA Drummond; Carol A Whiteis; Vivian Costa; Margaret Price; Christopher Benson; Michael J Welsh; Mark W Chapleau; Francois M Abboud
The ion channel ASIC2 is required for baroreceptor and autonomic control of the circulation.
Neuron 2009;64(6):885-97.
2009: Adam E Ziemann; Jason E Allen; Nader S Dahdaleh; Iuliia I Drebot; Matthew W Coryell; Amanda M Wunsch; Cynthia M Lynch; Frank M Faraci; Matthew A Howard; Michael J Welsh; John A Wemmie
The amygdala is a chemosensor that detects carbon dioxide and acidosis to elicit fear behavior.
Cell 2009;139(5):1012-21.
2009: Alok S Shah; Yehuda Ben-Shahar; Thomas O Moninger; Joel N Kline; Michael J Welsh
Motile cilia of human airway epithelia are chemosensory.
Science (New York, N.Y.) 2009;325(5944):1131-4.
2009: Yishan Sun; Lei Liu; Yehuda Ben-Shahar; JS Jacobs; Daniel F Eberl; Michael J Welsh
TRPA channels distinguish gravity sensing from hearing in Johnston's organ.
Proceedings of the National Academy of Sciences of the United States of America 2009;106(32):13606-11.
2009: Xiang-ming Zha; Vivian Costa; Anne Marie S Harding; Leah Reznikov; Christopher J Benson; Michael J Welsh
ASIC2 subunits target acid-sensing ion channels to the synapse via an association with PSD-95.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2009;29(26):8438-46.
2009: John E Butler; Kelly M Lager; I Splichal; D Francis; I Kacskovics; Marek Sinkora; Nancy Wertz; Jishan Sun; Y Zhao; W R Brown; R DeWald; S Dierks; Serge Muyldermans; J K Lunney; Paul B McCray; C S Rogers; Michael J Welsh; P Navarro; F Klobasa; F Habe; J Ramsoondar
The piglet as a model for B cell and immune system development.
Veterinary immunology and immunopathology 2009;128(1-3):147-70.
2009: Xiang-ming Zha; Runping Wang; Dan M Collier; Peter M Snyder; John A Wemmie; Michael J Welsh
Oxidant regulated inter-subunit disulfide bond formation between ASIC1a subunits.
Proceedings of the National Academy of Sciences of the United States of America 2009;106(9):3573-8.
2009: Andrea N Flynn; Omar A Itani; Thomas O Moninger; Michael J Welsh
Acute regulation of tight junction ion selectivity in human airway epithelia.
Proceedings of the National Academy of Sciences of the United States of America 2009;106(9):3591-6.
2009: Mikael K Schnizler; Katrin Schnizler; Xiang-ming Zha; Duane D Hall; John A Wemmie; Johannes Hell; Michael J Welsh
The cytoskeletal protein alpha-actinin regulates acid-sensing ion channel 1a through a C-terminal interaction.
The Journal of biological chemistry 2009;284(5):2697-705.
2008: Qian Dong; Christoph O Randak; Michael J Welsh
A mutation in CFTR modifies the effects of the adenylate kinase inhibitor Ap5A on channel gating.
Biophysical journal 2008;95(11):5178-85.
2008: Christopher S Rogers; David A Stoltz; David K Meyerholz; Lynda S Ostedgaard; Tatiana Rokhlina; Peter J Taft; Mark P Rogan; Alejandro A Pezzulo; Philip H Karp; Omar A Itani; Amanda C Kabel; Christine L Wohlford-Lenane; Greg J Davis; Robert A Hanfland; Tony L Smith; Melissa Samuel; David Wax; Clifton N Murphy; August Rieke; Kristin Whitworth; Aliye Uc; Timothy D Starner; Kim A Brogden; joel shilyansky; Paul B McCray; Joseph Zabner; Randall S Prather; Michael J Welsh
Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs.
Science (New York, N.Y.) 2008;321(5897):1837-41.
2008: Adam E Ziemann; Mikael Schnizler; Gregory W Albert; Meryl A Severson; Matthew A Howard; Michael J Welsh; John A Wemmie
Seizure termination by acidosis depends on ASIC1a.
Nature neuroscience 2008;11(7):816-22.
2008: Xingshen Sun; Ziying Yan; Yaling Yi; Ziyi Li; Diana Lei; Christopher S Rogers; Juan Chen; Yulong Zhang; Michael J Welsh; Gregory H Leno; John F Engelhardt
Adeno-associated virus-targeted disruption of the CFTR gene in cloned ferrets.
The Journal of clinical investigation 2008;118(4):1578-83.
2008: Christopher S Rogers; Yanhong Hao; Tatiana Rokhlina; Melissa Samuel; David A Stoltz; Yuhong Li; Elena Petroff; Daniel W Vermeer; Amanda C Kabel; Ziying Yan; Lee Spate; David Wax; Clifton N Murphy; August Rieke; Kristin Whitworth; Michael L Linville; Scott W Korte; John F Engelhardt; Michael J Welsh; Randall S Prather
Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer.
The Journal of clinical investigation 2008;118(4):1571-7.
2008: Alok S Shah; Sara L Farmen; Thomas O Moninger; Thomas R Businga; Michael P Andrews; Kevin Bugge; Charles C Searby; Darryl Nishimura; Kim A Brogden; Joel N Kline; Val C Sheffield; Michael J Welsh
Loss of Bardet-Biedl syndrome proteins alters the morphology and function of motile cilia in airway epithelia.
Proceedings of the National Academy of Sciences of the United States of America 2008;105(9):3380-5.
2008: Elena Yermolaieva Petroff; Margaret P Price; Vladislav Snitsarev; Huiyu Gong; Victoria Korovkina; Francois M Abboud; Michael J Welsh
Acid-sensing ion channels interact with and inhibit BK K+ channels.
Proceedings of the National Academy of Sciences of the United States of America 2008;105(8):3140-4.
2007: Manuel A Friese; Matthew J Craner; Ruth Etzensperger; Sandra Vergo; John A Wemmie; Michael J Welsh; Angela Vincent; Lars Fugger
Acid-sensing ion channel-1 contributes to axonal degeneration in autoimmune inflammation of the central nervous system.
Nature medicine 2007;13(12):1483-9.
2007: Christoph O Randak; Michael J Welsh
Role of CFTR's intrinsic adenylate kinase activity in gating of the Cl(-) channel.
Journal of bioenergetics and biomembranes 2007;39(5-6):473-9.
2007: Lei Liu; Yuhong Li; Runping Wang; Chong Yin; Qian Dong; Huey Hing; Changsoo Kim; Michael J Welsh
Drosophila hygrosensation requires the TRP channels water witch and nanchung.
Nature 2007;450(7167):294-8.
2007: Lynda S Ostedgaard; Christopher S Rogers; Qian Dong; Christoph O Randak; Daniel W Vermeer; Tatiana Rokhlina; Philip H Karp; Michael J Welsh
Processing and function of CFTR-DeltaF508 are species-dependent.
Proceedings of the National Academy of Sciences of the United States of America 2007;104(39):15370-5.
2007: Paola D Vermeer; Julia McHugh; Tatiana Rokhlina; Daniel W Vermeer; Joseph Zabner; Michael J Welsh
Vaccinia virus entry, exit, and interaction with differentiated human airway epithelia.
Journal of virology 2007;81(18):9891-9.
2007: Yehuda Ben-Shahar; Kishore Nannapaneni; Thomas L Casavant; Todd E Scheetz; Michael J Welsh
Eukaryotic operon-like transcription of functionally related genes in Drosophila.
Proceedings of the National Academy of Sciences of the United States of America 2007;104(1):222-7.
2006: Xiang-ming Zha; John A Wemmie; Steven H Green; Michael J Welsh
Acid-sensing ion channel 1a is a postsynaptic proton receptor that affects the density of dendritic spines.
Proceedings of the National Academy of Sciences of the United States of America 2006;103(44):16556-61.
2006: John A Wemmie; Margaret P Price; Michael J Welsh
Acid-sensing ion channels: advances, questions and therapeutic opportunities.
Trends in neurosciences 2006;29(10):578-86.
2006: Paola D Vermeer; Lacey Panko; Michael J Welsh; Joseph Zabner
erbB1 functions as a sensor of airway epithelial integrity by regulation of protein phosphatase 2A activity.
The Journal of biological chemistry 2006;281(3):1725-30.
2005: Sara L Farmen; Philip H Karp; Philip Ng; Donna J Palmer; David R Koehler; Jim Hu; Arthur L Beaudet; Joseph Zabner; Michael J Welsh
Gene transfer of CFTR to airway epithelia: low levels of expression are sufficient to correct Cl- transport and overexpression can generate basolateral CFTR.
American journal of physiology. Lung cellular and molecular physiology 2005;289(6):L1123-30.
2005: Christoph O Randak; Michael J Welsh
Adenylate kinase activity in ABC transporters.
The Journal of biological chemistry 2005;280(41):34385-8.
2005: Rana Dajani; Yulong Zhang; Peter J Taft; Sue M Travis; Timothy D Starner; Ansgar Olsen; Joseph Zabner; Michael J Welsh; John F Engelhardt
Lysozyme secretion by submucosal glands protects the airway from bacterial infection.
American journal of respiratory cell and molecular biology 2005;32(6):548-52.
2005: Lynda S Ostedgaard; Tatiana Rokhlina; Philip H Karp; Philip E Lashmit; Sandra Afione; Michael Schmidt; Joseph Zabner; Mark F Stinski; John A Chiorini; Michael J Welsh
A shortened adeno-associated virus expression cassette for CFTR gene transfer to cystic fibrosis airway epithelia.
Proceedings of the National Academy of Sciences of the United States of America 2005;102(8):2952-7.
2005: Allan L Berger; Christoph O Randak; Lynda S Ostedgaard; Philip H Karp; Daniel W Vermeer; Michael J Welsh
Curcumin stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.
The Journal of biological chemistry 2005;280(7):5221-6.
2005: Christoph O Randak; Michael J Welsh
ADP inhibits function of the ABC transporter cystic fibrosis transmembrane conductance regulator via its adenylate kinase activity.
Proceedings of the National Academy of Sciences of the United States of America 2005;102(6):2216-20.
2005: Allan L Berger; Mutsuhiro Ikuma; Michael J Welsh
Normal gating of CFTR requires ATP binding to both nucleotide-binding domains and hydrolysis at the second nucleotide-binding domain.
Proceedings of the National Academy of Sciences of the United States of America 2005;102(2):455-60.
2004: Alesia M Hruska-Hageman; Christopher J Benson; A Soren Leonard; Margaret P Price; Michael J Welsh
PSD-95 and Lin-7b interact with acid-sensing ion channel-3 and have opposite effects on H+- gated current.
The Journal of biological chemistry 2004;279(45):46962-8.
2004: Zhigang Xiong; Xiao-Man Zhu; Xiangping Chu; Manabu Minami; Jessica Hey; Wen-Li Wei; John F MacDonald; John A Wemmie; Margaret P Price; Michael J Welsh; Roger P. Simon
Neuroprotection in ischemia: blocking calcium-permeable acid-sensing ion channels.
Cell 2004;118(6):687-98.
2004: Candice C Askwith; John A Wemmie; Margaret P Price; Tatiana Rokhlina; Michael J Welsh
Acid-sensing ion channel 2 (ASIC2) modulates ASIC1 H+-activated currents in hippocampal neurons.
The Journal of biological chemistry 2004;279(18):18296-305.
2004: Olena Yermolaieva; A Soren Leonard; Mikael Schnizler; Francois M Abboud; Michael J Welsh
Extracellular acidosis increases neuronal cell calcium by activating acid-sensing ion channel 1a.
Proceedings of the National Academy of Sciences of the United States of America 2004;101(17):6752-7.
2004: Todd E Scheetz; Joseph Zabner; Michael J Welsh; Justin Coco; Mari Eyestone; Maria F Bonaldo; Tamara Kucaba; Thomas L Casavant; M Bento Soares; Paul B McCray
Large-scale gene discovery in human airway epithelia reveals novel transcripts.
Physiological genomics 2004;17(1):69-77.
2004: John A Wemmie; Matthew W Coryell; Candice C Askwith; Ejvis Lamani; A Soren Leonard; Curt D Sigmund; Michael J Welsh
Overexpression of acid-sensing ion channel 1a in transgenic mice increases acquired fear-related behavior.
Proceedings of the National Academy of Sciences of the United States of America 2004;101(10):3621-6.
2004: John H Lee; Su Min P Yi; Mary E Anderson; Kristi Berger; Michael J Welsh; Aloysius J Klingelhutz; Michelle A Ozbun
Propagation of infectious human papillomavirus type 16 by using an adenovirus and Cre/LoxP mechanism.
Proceedings of the National Academy of Sciences of the United States of America 2004;101(7):2094-9.
2003: Christoph O Randak; Michael J Welsh
An intrinsic adenylate kinase activity regulates gating of the ABC transporter CFTR.
Cell 2003;115(7):837-50.
2003: Kathleen A Sluka; Margaret P Price; Nicole M Breese; Cheryl L Stucky; John A Wemmie; Michael J Welsh
Chronic hyperalgesia induced by repeated acid injections in muscle is abolished by the loss of ASIC3, but not ASIC1.
Pain 2003;106(3):229-39.
2003: Joshua A Ainsley; Janette M Pettus; Dmitry Bosenko; Clare E Gerstein; Natalya Zinkevich; Michael G Anderson; Christopher M Adams; Michael J Welsh; Wayne A Johnson
Enhanced locomotion caused by loss of the Drosophila DEG/ENaC protein Pickpocket1.
Current biology : CB 2003;13(17):1557-63.
2003: Lei Liu; A Soren Leonard; David G Motto; Margaret A Feller; Margaret P Price; Wayne A Johnson; Michael J Welsh
Contribution of Drosophila DEG/ENaC genes to salt taste.
Neuron 2003;39(1):133-46.
2003: John A Wemmie; Candice C Askwith; Ejvis Lamani; Martin D Cassell; John H Freeman; Michael J Welsh
Acid-sensing ion channel 1 is localized in brain regions with high synaptic density and contributes to fear conditioning.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2003;23(13):5496-502.
2003: Jinghui Xie; Margaret P Price; John A Wemmie; Candice C Askwith; Michael J Welsh
ASIC3 and ASIC1 mediate FMRFamide-related peptide enhancement of H+-gated currents in cultured dorsal root ganglion neurons.
Journal of neurophysiology 2003;89(5):2459-65.
2003: Paola D Vermeer; Lisa A Einwalter; Thomas O Moninger; Tatiana Rokhlina; Jeffrey A Kern; Joseph Zabner; Michael J Welsh
Segregation of receptor and ligand regulates activation of epithelial growth factor receptor.
Nature 2003;422(6929):322-6.
2003: Lei Liu; Olena Yermolaieva; Wayne A Johnson; Francois M Abboud; Michael J Welsh
Identification and function of thermosensory neurons in Drosophila larvae.
Nature neuroscience 2003;6(3):267-73.
2003: Lynda S Ostedgaard; Christoph O Randak; Tatiana Rokhlina; Philip H Karp; Daniel W Vermeer; Katherine J Ashbourne Excoffon; Michael J Welsh
Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epithelia.
Proceedings of the National Academy of Sciences of the United States of America 2003;100(4):1937-42.
2003: A Soren Leonard; Olena Yermolaieva; Alesia M Hruska-Hageman; Candice C Askwith; Margaret P Price; John A Wemmie; Michael J Welsh
cAMP-dependent protein kinase phosphorylation of the acid-sensing ion channel-1 regulates its binding to the protein interacting with C-kinase-1.
Proceedings of the National Academy of Sciences of the United States of America 2003;100(4):2029-34.
2003: Lei Liu; Wayne A Johnson; Michael J Welsh
Drosophila DEG/ENaC pickpocket genes are expressed in the tracheal system, where they may be involved in liquid clearance.
Proceedings of the National Academy of Sciences of the United States of America 2003;100(4):2128-33.
2002: Robert Walters; Paul Freimuth; Thomas O Moninger; Ingrid Ganske; Joseph Zabner; Michael J Welsh
Adenovirus fiber disrupts CAR-mediated intercellular adhesion allowing virus escape.
Cell 2002;110(6):789-99.
2002: Jinghui Xie; Margaret P Price; Allan L Berger; Michael J Welsh
DRASIC contributes to pH-gated currents in large dorsal root ganglion sensory neurons by forming heteromultimeric channels.
Journal of neurophysiology 2002;87(6):2835-43.
2002: Pradeep K Singh; Matthew R Parsek; E Peter Greenberg; Michael J Welsh
A component of innate immunity prevents bacterial biofilm development.
Nature 2002;417(6888):552-5.
2002: John A Wemmie; Jianguo Chen; Candice C Askwith; Alesia M Hruska-Hageman; Margaret P Price; Brian C Nolan; Patrick G Yoder; Ejvis Lamani; Toshinori Hoshi; John H Freeman; Michael J Welsh
The acid-activated ion channel ASIC contributes to synaptic plasticity, learning, and memory.
Neuron 2002;34(3):463-77.
2002: Lynda S Ostedgaard; Joseph Zabner; Daniel W Vermeer; Tatiana Rokhlina; Philip H Karp; Arlene A Stecenko; Christoph O Randak; Michael J Welsh
CFTR with a partially deleted R domain corrects the cystic fibrosis chloride transport defect in human airway epithelia in vitro and in mouse nasal mucosa in vivo.
Proceedings of the National Academy of Sciences of the United States of America 2002;99(5):3093-8.
2002: Christopher J Benson; Jinghui Xie; John A Wemmie; Margaret P Price; Jillian M Henss; Michael J Welsh; Peter M Snyder
Heteromultimers of DEG/ENaC subunits form H+-gated channels in mouse sensory neurons.
Proceedings of the National Academy of Sciences of the United States of America 2002;99(4):2338-43.
2002: Brian C Schutte; Joseph P Mitros; Jennifer A Bartlett; Jesse D Walters; Hong Peng Jia; Michael J Welsh; Thomas L Casavant; Paul B McCray
Discovery of five conserved beta -defensin gene clusters using a computational search strategy.
Proceedings of the National Academy of Sciences of the United States of America 2002;99(4):2129-33.
2002: Alesia M Hruska-Hageman; John A Wemmie; Margaret P Price; Michael J Welsh
Interaction of the synaptic protein PICK1 (protein interacting with C kinase 1) with the non-voltage gated sodium channels BNC1 (brain Na+ channel 1) and ASIC (acid-sensing ion channel).
The Biochemical journal 2002;361(Pt 3):443-50.
2002: Michael J Welsh; Margaret P Price; Jinghui Xie
Biochemical basis of touch perception: mechanosensory function of degenerin/epithelial Na+ channels.
The Journal of biological chemistry 2002;277(4):2369-72.
2002: Allan L Berger; Mutsuhiro Ikuma; John F Hunt; Philip J Thomas; Michael J Welsh
Mutations that change the position of the putative gamma-phosphate linker in the nucleotide binding domains of CFTR alter channel gating.
The Journal of biological chemistry 2002;277(3):2125-31.
2002: Philip H Karp; Thomas O Moninger; S Pary Weber; Tamara S Nesselhauf; Janice Launspach; Joseph Zabner; Michael J Welsh
An in vitro model of differentiated human airway epithelia. Methods for establishing primary cultures.
Methods in molecular biology (Clifton, N.J.) 2002;188():115-37.
2001: Margaret P Price; SL McIlwrath; Jinghui Xie; C Cheng; J Qiao; Deirdre E Tarr; Kathleen A Sluka; Timothy Brennan; Gary R Lewin; Michael J Welsh
The DRASIC cation channel contributes to the detection of cutaneous touch and acid stimuli in mice.
Neuron 2001;32(6):1071-83.
2001: Su Min P Yi; R E Harson; Joseph Zabner; Michael J Welsh
Lectin binding and endocytosis at the apical surface of human airway epithelia.
Gene therapy 2001;8(24):1826-32.
2001: Robert Walters; W van't Hof; Su Min P Yi; M K Schroth; Joseph Zabner; R G Crystal; Michael J Welsh
Apical localization of the coxsackie-adenovirus receptor by glycosyl-phosphatidylinositol modification is sufficient for adenovirus-mediated gene transfer through the apical surface of human airway epithelia.
Journal of virology 2001;75(16):7703-11.
2001: Lawrence S Prince; Philip H Karp; Thomas O Moninger; Michael J Welsh
KGF alters gene expression in human airway epithelia: potential regulation of the inflammatory response.
Physiological genomics 2001;6(2):81-9.
2001: Michael J Welsh; J J Smith
cAMP stimulation of HCO3- secretion across airway epithelia.
JOP : Journal of the pancreas 2001;2(4 Suppl):291-3.
2001: Su Min P Yi; John H Lee; S Graham; Joseph Zabner; Michael J Welsh
Adenovirus calcium phosphate coprecipitates enhance squamous cell carcinoma gene transfer.
The Laryngoscope 2001;111(7):1290-6.
2001: Robert Walters; Su Min P Yi; Shaf Keshavjee; K E Brown; Michael J Welsh; John A Chiorini; Joseph Zabner
Binding of adeno-associated virus type 5 to 2,3-linked sialic acid is required for gene transfer.
The Journal of biological chemistry 2001;276(23):20610-6.
2001: HA Drummond; Michael J Welsh; Francois M Abboud
ENaC subunits are molecular components of the arterial baroreceptor complex.
Annals of the New York Academy of Sciences 2001;940():42-7.
2001: Candice C Askwith; Christopher J Benson; Michael J Welsh; Peter M Snyder
DEG/ENaC ion channels involved in sensory transduction are modulated by cold temperature.
Proceedings of the National Academy of Sciences of the United States of America 2001;98(11):6459-63.
2001: Lynda S Ostedgaard; O Baldursson; Michael J Welsh
Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by its R domain.
The Journal of biological chemistry 2001;276(11):7689-92.
2001: D J Hennager; Mutsuhiro Ikuma; Toshinori Hoshi; Michael J Welsh
A conditional probability analysis of cystic fibrosis transmembrane conductance regulator gating indicates that ATP has multiple effects during the gating cycle.
Proceedings of the National Academy of Sciences of the United States of America 2001;98(6):3594-9.
2001: Sue M Travis; Pradeep K Singh; Michael J Welsh
Antimicrobial peptides and proteins in the innate defense of the airway surface.
Current opinion in immunology 2001;13(1):89-95.
2001: O Baldursson; Lynda S Ostedgaard; Tatiana Rokhlina; JF Cotten; Michael J Welsh
Cystic fibrosis transmembrane conductance regulator Cl- channels with R domain deletions and translocations show phosphorylation-dependent and -independent activity.
The Journal of biological chemistry 2001;276(3):1904-10.
2000: HA Drummond; Francois M Abboud; Michael J Welsh
Localization of beta and gamma subunits of ENaC in sensory nerve endings in the rat foot pad.
Brain research 2000;884(1--2):1-12.
2000: O Baldursson; Herbert A Berger; Michael J Welsh
Contribution of R domain phosphoserines to the function of CFTR studied in Fischer rat thyroid epithelia.
American journal of physiology. Lung cellular and molecular physiology 2000;279(5):L835-41.
2000: Pradeep K Singh; Brian F Tack; Paul B McCray; Michael J Welsh
Synergistic and additive killing by antimicrobial factors found in human airway surface liquid.
American journal of physiology. Lung cellular and molecular physiology 2000;279(5):L799-805.
2000: Margaret P Price; Gary R Lewin; SL McIlwrath; C Cheng; Jinghui Xie; Paul A Heppenstall; Cheryl L Stucky; AG Mannsfeldt; Timothy Brennan; HA Drummond; J Qiao; Christopher J Benson; Deirdre E Tarr; Ronald Hrstka; Baoli Yang; Roger A Williamson; Michael J Welsh
The mammalian sodium channel BNC1 is required for normal touch sensation.
Nature 2000;407(6807):1007-11.
2000: Pradeep K Singh; Amy L Schaefer; Matthew R Parsek; Thomas O Moninger; Michael J Welsh; E Peter Greenberg
Quorum-sensing signals indicate that cystic fibrosis lungs are infected with bacterial biofilms.
Nature 2000;407(6805):762-4.
2000: Joseph Zabner; Michael Seiler; Janice Launspach; Philip H Karp; William R Kearney; Dwight C Look; J J Smith; Michael J Welsh
The osmolyte xylitol reduces the salt concentration of airway surface liquid and may enhance bacterial killing.
Proceedings of the National Academy of Sciences of the United States of America 2000;97(21):11614-9.
2000: Allan L Berger; Michael J Welsh
Differences between cystic fibrosis transmembrane conductance regulator and HisP in the interaction with the adenine ring of ATP.
The Journal of biological chemistry 2000;275(38):29407-12.
2000: Mutsuhiro Ikuma; Michael J Welsh
Regulation of CFTR Cl- channel gating by ATP binding and hydrolysis.
Proceedings of the National Academy of Sciences of the United States of America 2000;97(15):8675-80.
2000: Lynda S Ostedgaard; O Baldursson; Daniel W Vermeer; Michael J Welsh; A D Robertson
A functional R domain from cystic fibrosis transmembrane conductance regulator is predominantly unstructured in solution.
Proceedings of the National Academy of Sciences of the United States of America 2000;97(10):5657-62.
2000: Sue M Travis; N N Anderson; William R Forsyth; C Espiritu; B D Conway; E Peter Greenberg; Paul B McCray; Robert I Lehrer; Michael J Welsh; Brian F Tack
Bactericidal activity of mammalian cathelicidin-derived peptides.
Infection and immunity 2000;68(5):2748-55.
2000: Candice C Askwith; C Cheng; Mutsuhiro Ikuma; C Benson; Margaret P Price; Michael J Welsh
Neuropeptide FF and FMRFamide potentiate acid-evoked currents from sensory neurons and proton-gated DEG/ENaC channels.
Neuron 2000;26(1):133-41.
2000: Paola T Drapkin; Catherine R O'Riordan; Su Min P Yi; John A Chiorini; J Cardella; Joseph Zabner; Michael J Welsh
Targeting the urokinase plasminogen activator receptor enhances gene transfer to human airway epithelia.
The Journal of clinical investigation 2000;105(5):589-96.
2000: Robert Walters; Dongsheng Duan; John F Engelhardt; Michael J Welsh
Incorporation of adeno-associated virus in a calcium phosphate coprecipitate improves gene transfer to airway epithelia in vitro and in vivo.
Journal of virology 2000;74(1):535-40.
1999: Lawrence S Prince; Janice Launspach; David S Geller; Richard P Lifton; J H Pratt; Joseph Zabner; Michael J Welsh
Absence of amiloride-sensitive sodium absorption in the airway of an infant with pseudohypoaldosteronism.
The Journal of pediatrics 1999;135(6):786-9.
1999: Michael J Welsh
Gene transfer for cystic fibrosis.
The Journal of clinical investigation 1999;104(9):1165-6.
1999: J H Lee; T J Baker; L K Mahal; Joseph Zabner; Carolyn R Bertozzi; David F Wiemer; Michael J Welsh
Engineering novel cell surface receptors for virus-mediated gene transfer.
The Journal of biological chemistry 1999;274(31):21878-84.
1999: Paul B McCray; Joseph Zabner; Hong Peng Jia; Michael J Welsh; P S Thorne
Efficient killing of inhaled bacteria in DeltaF508 mice: role of airway surface liquid composition.
The American journal of physiology 1999;277(1 Pt 1):L183-90.
1999: Lynda S Ostedgaard; B Zeiher; Michael J Welsh
Processing of CFTR bearing the P574H mutation differs from wild-type and deltaF508-CFTR.
Journal of cell science 1999;112 ( Pt 13)():2091-8.
1999: Michael J Welsh; Joseph Zabner
Cationic lipid mediated gene transfer of CFTR: safety of a single administration to the nasal epithelia.
Human gene therapy 1999;10(9):1559-72.
1999: Lawrence S Prince; Michael J Welsh
Effect of subunit composition and Liddle's syndrome mutations on biosynthesis of ENaC.
The American journal of physiology 1999;276(6 Pt 1):C1346-51.
1999: Christopher M Adams; Peter M Snyder; Michael J Welsh
Paradoxical stimulation of a DEG/ENaC channel by amiloride.
The Journal of biological chemistry 1999;274(22):15500-4.
1999: Sue M Travis; Barbara-Ann Conway; Joseph Zabner; J J Smith; N N Anderson; Pradeep K Singh; E Peter Greenberg; Michael J Welsh
Activity of abundant antimicrobials of the human airway.
American journal of respiratory cell and molecular biology 1999;20(5):872-9.
1999: Robert Walters; T Grunst; J M Bergelson; R W Finberg; Michael J Welsh; Joseph Zabner
Basolateral localization of fiber receptors limits adenovirus infection from the apical surface of airway epithelia.
The Journal of biological chemistry 1999;274(15):10219-26.
1999: John H Lee; Michael J Welsh
Enhancement of calcium phosphate-mediated transfection by inclusion of adenovirus in coprecipitates.
Gene therapy 1999;6(4):676-82.
1999: Scott M Graham; Janice Launspach; Michael J Welsh; Joseph Zabner
Sequential magnetic resonance imaging analysis of the maxillary sinuses: implications for a model of gene therapy in cystic fibrosis.
The Journal of laryngology and otology 1999;113(4):329-35.
1999: John H Lee; Joseph Zabner; Michael J Welsh
Delivery of an adenovirus vector in a calcium phosphate coprecipitate enhances the therapeutic index of gene transfer to airway epithelia.
Human gene therapy 1999;10(4):603-13.
1999: Christopher M Adams; Margaret P Price; Peter M Snyder; Michael J Welsh
Tetraethylammonium block of the BNC1 channel.
Biophysical journal 1999;76(3):1377-83.
1999: Miguel Chillon; Assumpció Bosch; Joseph Zabner; Lane K Law; D Armentano; Michael J Welsh; Beverly L Davidson
Group D adenoviruses infect primary central nervous system cells more efficiently than those from group C.
Journal of virology 1999;73(3):2537-40.
1999: JF Cotten; Michael J Welsh
Cystic fibrosis-associated mutations at arginine 347 alter the pore architecture of CFTR. Evidence for disruption of a salt bridge.
The Journal of biological chemistry 1999;274(9):5429-35.
1999: Fiona J McDonald; Baoli Yang; Ronald Hrstka; HA Drummond; Deirdre E Tarr; Paul B McCray; John B Stokes; Michael J Welsh; Roger A Williamson
Disruption of the beta subunit of the epithelial Na+ channel in mice: hyperkalemia and neonatal death associated with a pseudohypoaldosteronism phenotype.
Proceedings of the National Academy of Sciences of the United States of America 1999;96(4):1727-31.
1998: Michael J Welsh; A D Robertson; Lynda S Ostedgaard
Structural biology. The ABC of a versatile engine.
Nature 1998;396(6712):623-4.
1998: Lawrence S Prince; Michael J Welsh
Cell surface expression and biosynthesis of epithelial Na+ channels.
The Biochemical journal 1998;336 ( Pt 3)():705-10.
1998: Pradeep K Singh; Hong Peng Jia; KR Wiles; J Hesselberth; Lide Liu; Barbara-Ann Conway; E Peter Greenberg; Erika V Valore; Michael J Welsh; Tomas Ganz; Brian F Tack; Paul B McCray
Production of beta-defensins by human airway epithelia.
Proceedings of the National Academy of Sciences of the United States of America 1998;95(25):14961-6.
1998: HA Drummond; Margaret P Price; Michael J Welsh; Francois M Abboud
A molecular component of the arterial baroreceptor mechanotransducer.
Neuron 1998;21(6):1435-41.
1998: JF Cotten; Michael J Welsh
Covalent modification of the nucleotide binding domains of cystic fibrosis transmembrane conductance regulator.
The Journal of biological chemistry 1998;273(48):31873-9.
1998: Christopher M Adams; Peter M Snyder; Margaret P Price; Michael J Welsh
Protons activate brain Na+ channel 1 by inducing a conformational change that exposes a residue associated with neurodegeneration.
The Journal of biological chemistry 1998;273(46):30204-7.
1998: K Toyoda; H Ooboshi; Yi Chu; A Fasbender; Beverly L Davidson; Michael J Welsh; Donald D Heistad
Cationic polymer and lipids enhance adenovirus-mediated gene transfer to rabbit carotid artery.
Stroke; a journal of cerebral circulation 1998;29(10):2181-8.
1998: T Hautala; T Grunst; A Fabrega; Paul Freimuth; Michael J Welsh
An interaction between penton base and alpha v integrins plays a minimal role in adenovirus-mediated gene transfer to hepatocytes in vitro and in vivo.
Gene therapy 1998;5(9):1259-64.
1998: Joseph Zabner; J J Smith; Philip H Karp; Jonathan H Widdicombe; Michael J Welsh
Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro.
Molecular cell 1998;2(3):397-403.
1998: C Cheng; Lawrence S Prince; Peter M Snyder; Michael J Welsh
Assembly of the epithelial Na+ channel evaluated using sucrose gradient sedimentation analysis.
The Journal of biological chemistry 1998;273(35):22693-700.
1998: RA Hall; Lynda S Ostedgaard; Richard T Premont; Jeremy T Blitzer; N Rahman; Michael J Welsh; Robert J Lefkowitz
A C-terminal motif found in the beta2-adrenergic receptor, P2Y1 receptor and cystic fibrosis transmembrane conductance regulator determines binding to the Na+/H+ exchanger regulatory factor family of PDZ proteins.
Proceedings of the National Academy of Sciences of the United States of America 1998;95(15):8496-501.
1998: M Chillón; John H Lee; A Fasbender; Michael J Welsh
Adenovirus complexed with polyethylene glycol and cationic lipid is shielded from neutralizing antibodies in vitro.
Gene therapy 1998;5(7):995-1002.
1998: A Fasbender; John H Lee; Robert Walters; Thomas O Moninger; Joseph Zabner; Michael J Welsh
Incorporation of adenovirus in calcium phosphate precipitates enhances gene transfer to airway epithelia in vitro and in vivo.
The Journal of clinical investigation 1998;102(1):184-93.
1998: Herbert A Berger; Sue M Travis; Michael J Welsh
Fluoride stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.
The American journal of physiology 1998;274(3 Pt 1):L305-12.
1998: Christopher M Adams; Michael G Anderson; David G Motto; Margaret P Price; Wayne A Johnson; Michael J Welsh
Ripped pocket and pickpocket, novel Drosophila DEG/ENaC subunits expressed in early development and in mechanosensory neurons.
The Journal of cell biology 1998;140(1):143-52.
1998: Peter M Snyder; C Cheng; Lawrence S Prince; J C Rogers; Michael J Welsh
Electrophysiological and biochemical evidence that DEG/ENaC cation channels are composed of nine subunits.
The Journal of biological chemistry 1998;273(2):681-4.
1997: A Fasbender; Joseph Zabner; B G Zeiher; Michael J Welsh
A low rate of cell proliferation and reduced DNA uptake limit cationic lipid-mediated gene transfer to primary cultures of ciliated human airway epithelia.
Gene therapy 1997;4(11):1173-80.
1997: Christopher M Adams; Peter M Snyder; Michael J Welsh
Interactions between subunits of the human epithelial sodium channel.
The Journal of biological chemistry 1997;272(43):27295-300.
1997: JF Cotten; Michael J Welsh
Covalent modification of the regulatory domain irreversibly stimulates cystic fibrosis transmembrane conductance regulator.
The Journal of biological chemistry 1997;272(41):25617-22.
1997: H Ishihara; Michael J Welsh
Block by MOPS reveals a conformation change in the CFTR pore produced by ATP hydrolysis.
The American journal of physiology 1997;273(4 Pt 1):C1278-89.
1997: Sue M Travis; Herbert A Berger; Michael J Welsh
Protein phosphatase 2C dephosphorylates and inactivates cystic fibrosis transmembrane conductance regulator.
Proceedings of the National Academy of Sciences of the United States of America 1997;94(20):11055-60.
1997: Michael C Winter; Michael J Welsh
Stimulation of CFTR activity by its phosphorylated R domain.
Nature 1997;389(6648):294-6.
1997: Joseph Zabner; Seng Cheng; DP Meeker; Janice Launspach; Robert H Balfour; Michael A Perricone; James E Morris; John Marshall; A Fasbender; A E Smith; Michael J Welsh
Comparison of DNA-lipid complexes and DNA alone for gene transfer to cystic fibrosis airway epithelia in vivo.
The Journal of clinical investigation 1997;100(6):1529-37.
1997: Joseph Zabner; Paul Freimuth; A Puga; A Fabrega; Michael J Welsh
Lack of high affinity fiber receptor activity explains the resistance of ciliated airway epithelia to adenovirus infection.
The Journal of clinical investigation 1997;100(5):1144-9.
1997: Sue M Travis; Michael J Welsh
PP2C gamma: a human protein phosphatase with a unique acidic domain.
FEBS letters 1997;412(3):415-9.
1997: A Fasbender; John Marshall; Thomas O Moninger; T Grunst; Seng Cheng; Michael J Welsh
Effect of co-lipids in enhancing cationic lipid-mediated gene transfer in vitro and in vivo.
Gene therapy 1997;4(7):716-25.
1997: MG Muhonen; H Ooboshi; Michael J Welsh; Beverly L Davidson; Donald D Heistad
Gene transfer to cerebral blood vessels after subarachnoid hemorrhage.
Stroke; a journal of cerebral circulation 1997;28(4):822-8; discussion 828-9.
1997: A Fasbender; Joseph Zabner; M Chillón; Thomas O Moninger; A P Puga; Beverly L Davidson; Michael J Welsh
Complexes of adenovirus with polycationic polymers and cationic lipids increase the efficiency of gene transfer in vitro and in vivo.
The Journal of biological chemistry 1997;272(10):6479-89.
1997: Lynda S Ostedgaard; D P Rich; L G DeBerg; Michael J Welsh
Association of domains within the cystic fibrosis transmembrane conductance regulator.
Biochemistry 1997;36(6):1287-94.
1996: Margaret P Price; H Ishihara; D N Sheppard; Michael J Welsh
Function of Xenopus cystic fibrosis transmembrane conductance regulator (CFTR) Cl channels and use of human-Xenopus chimeras to investigate the pore properties of CFTR.
The Journal of biological chemistry 1996;271(41):25184-91.
1996: Joseph Zabner; B G Zeiher; E Friedman; Michael J Welsh
Adenovirus-mediated gene transfer to ciliated airway epithelia requires prolonged incubation time.
Journal of virology 1996;70(10):6994-7003.
1996: JF Cotten; Lynda S Ostedgaard; M R Carson; Michael J Welsh
Effect of cystic fibrosis-associated mutations in the fourth intracellular loop of cystic fibrosis transmembrane conductance regulator.
The Journal of biological chemistry 1996;271(35):21279-84.
1996: K Matsushita; Paul B McCray; Rita D Sigmund; Michael J Welsh; John B Stokes
Localization of epithelial sodium channel subunit mRNAs in adult rat lung by in situ hybridization.
The American journal of physiology 1996;271(2 Pt 1):L332-9.
1996: D N Sheppard; Sue M Travis; H Ishihara; Michael J Welsh
Contribution of proline residues in the membrane-spanning domains of cystic fibrosis transmembrane conductance regulator to chloride channel function.
The Journal of biological chemistry 1996;271(25):14995-5001.
1996: Joseph Zabner; S C Wadsworth; AE Smith; Michael J Welsh
Adenovirus-mediated generation of cAMP-stimulated Cl- transport in cystic fibrosis airway epithelia in vitro: effect of promoter and administration method.
Gene therapy 1996;3(5):458-65.
1996: J J Smith; Sue M Travis; E Peter Greenberg; Michael J Welsh
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.
Cell 1996;85(2):229-36.
1996: Margaret P Price; Peter M Snyder; Michael J Welsh
Cloning and expression of a novel human brain Na+ channel.
The Journal of biological chemistry 1996;271(14):7879-82.
1996: Sylvain DeLisle; O Blondel; F J Longo; W E Schnabel; G I Bell; Michael J Welsh
Expression of inositol 1,4,5-trisphosphate receptors changes the Ca2+ signal of Xenopus oocytes.
The American journal of physiology 1996;270(4 Pt 1):C1255-61.
1996: J L Teem; M R Carson; Michael J Welsh
Mutation of R555 in CFTR-delta F508 enhances function and partially corrects defective processing.
Receptors & channels 1996;4(1):63-72.
1995: Peter M Snyder; Margaret P Price; F J McDonald; Christopher M Adams; Kenneth A Volk; B G Zeiher; John B Stokes; Michael J Welsh
Mechanism by which Liddle's syndrome mutations increase activity of a human epithelial Na+ channel.
Cell 1995;83(6):969-78.
1995: Kenneth A Volk; Rita D Sigmund; Peter M Snyder; F J McDonald; Michael J Welsh; John B Stokes
rENaC is the predominant Na+ channel in the apical membrane of the rat renal inner medullary collecting duct.
The Journal of clinical investigation 1995;96(6):2748-57.
1995: M R Carson; Michael J Welsh
Structural and functional similarities between the nucleotide-binding domains of CFTR and GTP-binding proteins.
Biophysical journal 1995;69(6):2443-8.
1995: F J McDonald; Michael J Welsh
Binding of the proline-rich region of the epithelial Na+ channel to SH3 domains and its association with specific cellular proteins.
The Biochemical journal 1995;312 ( Pt 2)():491-7.
1995: B G Zeiher; E Eichwald; Joseph Zabner; J J Smith; A P Puga; Paul B McCray; Mario R Capecchi; Michael J Welsh; Kirk R Thomas
A mouse model for the delta F508 allele of cystic fibrosis.
The Journal of clinical investigation 1995;96(4):2051-64.
1995: M R Carson; Michael C Winter; Sue M Travis; Michael J Welsh
Pyrophosphate stimulates wild-type and mutant cystic fibrosis transmembrane conductance regulator Cl- channels.
The Journal of biological chemistry 1995;270(35):20466-72.
1995: Joseph Zabner; A Fasbender; Thomas O Moninger; K A Poellinger; Michael J Welsh
Cellular and molecular barriers to gene transfer by a cationic lipid.
The Journal of biological chemistry 1995;270(32):18997-9007.
1995: A Fasbender; Joseph Zabner; Michael J Welsh
Optimization of cationic lipid-mediated gene transfer to airway epithelia.
The American journal of physiology 1995;269(1 Pt 1):L45-51.
1995: H Ooboshi; Michael J Welsh; C D Rios; Beverly L Davidson; Donald D Heistad
Adenovirus-mediated gene transfer in vivo to cerebral blood vessels and perivascular tissue.
Circulation research 1995;77(1):7-13.
1995: Sylvain DeLisle; G W Mayr; Michael J Welsh
Inositol phosphate structural requisites for Ca2+ influx.
The American journal of physiology 1995;268(6 Pt 1):C1485-91.
1995: Paul B McCray; K Armstrong; Joseph Zabner; D W Miller; Gary A Koretzky; L Couture; Josée Robillard; AE Smith; Michael J Welsh
Adenoviral-mediated gene transfer to fetal pulmonary epithelia in vitro and in vivo.
The Journal of clinical investigation 1995;95(6):2620-32.
1995: F J McDonald; Margaret P Price; Peter M Snyder; Michael J Welsh
Cloning and expression of the beta- and gamma-subunits of the human epithelial sodium channel.
The American journal of physiology 1995;268(5 Pt 1):C1157-63.
1995: Seng Cheng; S L Fang; Joseph Zabner; John Marshall; S Piraino; S C Schiavi; Douglas M Jefferson; Michael J Welsh; AE Smith
Functional activation of the cystic fibrosis trafficking mutant delta F508-CFTR by overexpression.
The American journal of physiology 1995;268(4 Pt 1):L615-24.
1995: D N Sheppard; Lynda S Ostedgaard; Michael C Winter; Michael J Welsh
Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency.
The EMBO journal 1995;14(5):876-83.
1995: Michael J Welsh; Joseph Zabner; Scott M Graham; AE Smith; R Moscicki; S Wadsworth
Adenovirus-mediated gene transfer for cystic fibrosis: Part A. Safety of dose and repeat administration in the nasal epithelium. Part B. Clinical efficacy in the maxillary sinus.
Human gene therapy 1995;6(2):205-18.
1995: M R Carson; Sue M Travis; Michael J Welsh
The two nucleotide-binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) have distinct functions in controlling channel activity.
The Journal of biological chemistry 1995;270(4):1711-7.
1994: M R Carson; Sue M Travis; Michael C Winter; D N Sheppard; Michael J Welsh
Phosphate stimulates CFTR Cl- channels.
Biophysical journal 1994;67(5):1867-75.
1994: Peter M Snyder; F J McDonald; John B Stokes; Michael J Welsh
Membrane topology of the amiloride-sensitive epithelial sodium channel.
The Journal of biological chemistry 1994;269(39):24379-83.
1994: T Takahashi; K Matsushita; Michael J Welsh; John B Stokes
Effect of cAMP on intracellular and extracellular ATP content of Cl- -secreting epithelia and 3T3 fibroblasts.
The Journal of biological chemistry 1994;269(27):17853-7.
1994: F J McDonald; Peter M Snyder; Paul B McCray; Michael J Welsh
Cloning, expression, and tissue distribution of a human amiloride-sensitive Na+ channel.
The American journal of physiology 1994;266(6 Pt 1):L728-34.
1994: Michael C Winter; D N Sheppard; M R Carson; Michael J Welsh
Effect of ATP concentration on CFTR Cl- channels: a kinetic analysis of channel regulation.
Biophysical journal 1994;66(5):1398-403.
1994: Joseph Zabner; L A Couture; AE Smith; Michael J Welsh
Correction of cAMP-stimulated fluid secretion in cystic fibrosis airway epithelia: efficiency of adenovirus-mediated gene transfer in vitro.
Human gene therapy 1994;5(5):585-93.
1994: D N Sheppard; M R Carson; Lynda S Ostedgaard; Gerene M Denning; Michael J Welsh
Expression of cystic fibrosis transmembrane conductance regulator in a model epithelium.
The American journal of physiology 1994;266(4 Pt 1):L405-13.
1994: D N Sheppard; Lynda S Ostedgaard; D P Rich; Michael J Welsh
The amino-terminal portion of CFTR forms a regulated Cl- channel.
Cell 1994;76(6):1091-8.
1994: J J Smith; P H Karp; Michael J Welsh
Defective fluid transport by cystic fibrosis airway epithelia.
The Journal of clinical investigation 1994;93(3):1307-11.
1994: Sylvain DeLisle; T Radenberg; M R Wintermantel; C Tietz; Jan B Parys; Didier Pittet; Michael J Welsh; G W Mayr
Second messenger specificity of the inositol trisphosphate receptor: reappraisal based on novel inositol phosphates.
The American journal of physiology 1994;266(2 Pt 1):C429-36.
1994: Michael J Welsh; AE Smith; Joseph Zabner; D P Rich; Scott M Graham; Richard J Gregory; B M Pratt; R A Moscicki
Cystic fibrosis gene therapy using an adenovirus vector: in vivo safety and efficacy in nasal epithelium.
Human gene therapy 1994;5(2):209-19.
1994: John Marshall; S Fang; Lynda S Ostedgaard; C R O'Riordan; D Ferrara; J F Amara; H Hoppe; Ronald K Scheule; Michael J Welsh; AE Smith
Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro.
The Journal of biological chemistry 1994;269(4):2987-95.
1994: Herbert A Berger; D P Rich; Seng Cheng; Sue M Travis; M Saxena; AE Smith; Michael J Welsh
Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by negative charge in the R domain.
The Japanese journal of physiology 1994;44 Suppl 2():S167-72.
1993: D N Sheppard; Michael J Welsh
Inhibition of the cystic fibrosis transmembrane conductance regulator by ATP-sensitive K+ channel regulators.
Annals of the New York Academy of Sciences 1993;707():275-84.
1993: Paul B McCray; J D Bettencourt; J Bastacky; Gerene M Denning; Michael J Welsh
Expression of CFTR and a cAMP-stimulated chloride secretory current in cultured human fetal alveolar epithelial cells.
American journal of respiratory cell and molecular biology 1993;9(6):578-85.
1993: Joseph Zabner; L A Couture; Richard J Gregory; Scott M Graham; AE Smith; Michael J Welsh
Adenovirus-mediated gene transfer transiently corrects the chloride transport defect in nasal epithelia of patients with cystic fibrosis.
Cell 1993;75(2):207-16.
1993: D P Rich; Herbert A Berger; Seng Cheng; Sue M Travis; M Saxena; AE Smith; Michael J Welsh
Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by negative charge in the R domain.
The Journal of biological chemistry 1993;268(27):20259-67.
1993: D P Rich; L A Couture; L M Cardoza; V M Guiggio; Donna Armentano; P C Espino; K Hehir; Michael J Welsh; AE Smith; Richard J Gregory
Development and analysis of recombinant adenoviruses for gene therapy of cystic fibrosis.
Human gene therapy 1993;4(4):461-76.
1993: Sue M Travis; M R Carson; D R Ries; Michael J Welsh
Interaction of nucleotides with membrane-associated cystic fibrosis transmembrane conductance regulator.
The Journal of biological chemistry 1993;268(21):15336-9.
1993: Michael J Welsh; AE Smith
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis.
Cell 1993;73(7):1251-4.
1993: M R Carson; Michael J Welsh
5'-Adenylylimidodiphosphate does not activate CFTR chloride channels in cell-free patches of membrane.
The American journal of physiology 1993;265(1 Pt 1):L27-32.
1993: J L Teem; Herbert A Berger; Lynda S Ostedgaard; D P Rich; L C Tsui; Michael J Welsh
Identification of revertants for the cystic fibrosis delta F508 mutation using STE6-CFTR chimeras in yeast.
Cell 1993;73(2):335-46.
1993: J J Smith; Michael J Welsh
Fluid and electrolyte transport by cultured human airway epithelia.
The Journal of clinical investigation 1993;91(4):1590-7.
1993: D N Sheppard; D P Rich; Lynda S Ostedgaard; Richard J Gregory; AE Smith; Michael J Welsh
Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties.
Nature 1993;362(6416):160-4.
1993: Herbert A Berger; Sue M Travis; Michael J Welsh
Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by specific protein kinases and protein phosphatases.
The Journal of biological chemistry 1993;268(3):2037-47.
1993: Michael J Welsh; Matthew Peter Anderson
Regulation of the cystic fibrosis transmembrane conductance regulator chloride channel by MgATP.
Society of General Physiologists series 1993;48():119-27.
1993: D P Rich; Richard J Gregory; Seng Cheng; AE Smith; Michael J Welsh
Effect of deletion mutations on the function of CFTR chloride channels.
Receptors & channels 1993;1(3):221-32.
1993: Michael J Welsh; Gerene M Denning; Lynda S Ostedgaard; Matthew Peter Anderson
Dysfunction of CFTR bearing the delta F508 mutation.
Journal of cell science. Supplement 1993;17():235-9.
1992: Lynda S Ostedgaard; Michael J Welsh
Partial purification of the cystic fibrosis transmembrane conductance regulator.
The Journal of biological chemistry 1992;267(36):26142-9.
1992: D N Sheppard; Michael J Welsh
Effect of ATP-sensitive K+ channel regulators on cystic fibrosis transmembrane conductance regulator chloride currents.
The Journal of general physiology 1992;100(4):573-91.
1992: Matthew Peter Anderson; Michael J Welsh
Regulation by ATP and ADP of CFTR chloride channels that contain mutant nucleotide-binding domains.
Science (New York, N.Y.) 1992;257(5077):1701-4.
1992: Jan B Parys; S W Sernett; Sylvain DeLisle; Peter M Snyder; Michael J Welsh; Kevin P Campbell
Isolation, characterization, and localization of the inositol 1,4,5-trisphosphate receptor protein in Xenopus laevis oocytes.
The Journal of biological chemistry 1992;267(26):18776-82.
1992: Gerene M Denning; Matthew Peter Anderson; J F Amara; John Marshall; AE Smith; Michael J Welsh
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive.
Nature 1992;358(6389):761-4.
1992: Gerene M Denning; Lynda S Ostedgaard; Michael J Welsh
Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia.
The Journal of cell biology 1992;118(3):551-9.
1992: Lynda S Ostedgaard; D Michael Shasby; Michael J Welsh
Staphylococcus aureus alpha-toxin permeabilizes the basolateral membrane of a Cl(-)-secreting epithelium.
The American journal of physiology 1992;263(1 Pt 1):L104-12.
1992: Matthew Peter Anderson; D N Sheppard; Herbert A Berger; Michael J Welsh
Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia.
The American journal of physiology 1992;263(1 Pt 1):L1-14.
1992: Sylvain DeLisle; Didier Pittet; B V Potter; P D Lew; Michael J Welsh
InsP3 and Ins(1,3,4,5)P4 act in synergy to stimulate influx of extracellular Ca2+ in Xenopus oocytes.
The American journal of physiology 1992;262(6 Pt 1):C1456-63.
1992: B C Tilly; Michael C Winter; Lynda S Ostedgaard; C O'Riordan; AE Smith; Michael J Welsh
Cyclic AMP-dependent protein kinase activation of cystic fibrosis transmembrane conductance regulator chloride channels in planar lipid bilayers.
The Journal of biological chemistry 1992;267(14):9470-3.
1992: Michael J Welsh; Matthew Peter Anderson; D P Rich; Herbert A Berger; Gerene M Denning; Lynda S Ostedgaard; D N Sheppard; Seng Cheng; Richard J Gregory; AE Smith
Cystic fibrosis transmembrane conductance regulator: a chloride channel with novel regulation.
Neuron 1992;8(5):821-9.
1992: Sylvain DeLisle; Michael J Welsh
Inositol trisphosphate is required for the propagation of calcium waves in Xenopus oocytes.
The Journal of biological chemistry 1992;267(12):7963-6.
1992: J J Smith; Michael J Welsh
cAMP stimulates bicarbonate secretion across normal, but not cystic fibrosis airway epithelia.
The Journal of clinical investigation 1992;89(4):1148-53.
1992: Gerene M Denning; Lynda S Ostedgaard; Seng Cheng; AE Smith; Michael J Welsh
Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia.
The Journal of clinical investigation 1992;89(1):339-49.
1991: Matthew Peter Anderson; Herbert A Berger; D P Rich; Richard J Gregory; AE Smith; Michael J Welsh
Nucleoside triphosphates are required to open the CFTR chloride channel.
Cell 1991;67(4):775-84.
1991: Herbert A Berger; Matthew Peter Anderson; Richard J Gregory; S Thompson; Paul W Howard; Richard A Maurer; R Mulligan; AE Smith; Michael J Welsh
Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channel.
The Journal of clinical investigation 1991;88(4):1422-31.
1991: Seng Cheng; D P Rich; John Marshall; Richard J Gregory; Michael J Welsh; AE Smith
Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel.
Cell 1991;66(5):1027-36.
1991: Richard J Gregory; D P Rich; Seng Cheng; D W Souza; S Paul; P Manavalan; Matthew Peter Anderson; Michael J Welsh; AE Smith
Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2.
Molecular and cellular biology 1991;11(8):3886-93.
1991: Matthew Peter Anderson; Michael J Welsh
Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia.
Proceedings of the National Academy of Sciences of the United States of America 1991;88(14):6003-7.
1991: Gerene M Denning; Michael J Welsh
Polarized distribution of bradykinin receptors on airway epithelial cells and independent coupling to second messenger pathways.
The Journal of biological chemistry 1991;266(20):12932-8.
1991: D P Rich; Richard J Gregory; Matthew Peter Anderson; P Manavalan; AE Smith; Michael J Welsh
Effect of deleting the R domain on CFTR-generated chloride channels.
Science (New York, N.Y.) 1991;253(5016):205-7.
1991: Matthew Peter Anderson; Richard J Gregory; S Thompson; D W Souza; S Paul; R C Mulligan; AE Smith; Michael J Welsh
Demonstration that CFTR is a chloride channel by alteration of its anion selectivity.
Science (New York, N.Y.) 1991;253(5016):202-5.
1991: Herbert A Berger; Michael J Welsh
Electrolyte transport in the lungs.
Hospital practice (Office ed.) 1991;26(3):53-9.
1991: Matthew Peter Anderson; D P Rich; Richard J Gregory; AE Smith; Michael J Welsh
Generation of cAMP-activated chloride currents by expression of CFTR.
Science (New York, N.Y.) 1991;251(4994):679-82.
1990: Ross D Feldman; A Brotherton; Michael J Welsh
Beta-adrenergic-mediated Cl secretion: evidence for additional non-cAMP-dependent pathway of effect.
The American journal of physiology 1990;259(6 Pt 1):L426-31.
1990: Douglas M Jefferson; J D Valentich; F C Marini; Shelley A Grubman; M C Iannuzzi; H L Dorkin; M Li; K W Klinger; Michael J Welsh
Expression of normal and cystic fibrosis phenotypes by continuous airway epithelial cell lines.
The American journal of physiology 1990;259(6 Pt 1):L496-505.
1990: J P Clancy; J D McCann; Michael J Welsh
Evidence that Ca2(+)-dependent activation of airway epithelia Cl- channels is not dependent on phosphorylation.
The American journal of physiology 1990;259(6 Pt 1):L410-4.
1990: Richard J Gregory; Seng Cheng; D P Rich; John Marshall; S Paul; K Hehir; Lynda S Ostedgaard; K W Klinger; Michael J Welsh; AE Smith
Expression and characterization of the cystic fibrosis transmembrane conductance regulator.
Nature 1990;347(6291):382-6.
1990: D P Rich; Matthew Peter Anderson; Richard J Gregory; Seng Cheng; S Paul; Douglas M Jefferson; J D McCann; K W Klinger; AE Smith; Michael J Welsh
Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells.
Nature 1990;347(6291):358-63.
1990: Matthew Peter Anderson; Michael J Welsh
Fatty acids inhibit apical membrane chloride channels in airway epithelia.
Proceedings of the National Academy of Sciences of the United States of America 1990;87(18):7334-8.
1990: M Li; J D McCann; Michael J Welsh
Apical membrane Cl- channels in airway epithelia: anion selectivity and effect of an inhibitor.
The American journal of physiology 1990;259(2 Pt 1):C295-301.
1990: Sylvain DeLisle; K H Krause; Gerene M Denning; B V Potter; Michael J Welsh
Effect of inositol trisphosphate and calcium on oscillating elevations of intracellular calcium in Xenopus oocytes.
The Journal of biological chemistry 1990;265(20):11726-30.
1990: Matthew Peter Anderson; Michael J Welsh
Isoproterenol, cAMP, and bradykinin stimulate diacylglycerol production in airway epithelium.
The American journal of physiology 1990;258(6 Pt 1):L294-300.
1990: J J Smith; J D McCann; Michael J Welsh
Bradykinin stimulates airway epithelial Cl- secretion via two second messenger pathways.
The American journal of physiology 1990;258(6 Pt 1):L369-77.
1990: J D McCann; Michael J Welsh
Basolateral K+ channels in airway epithelia. II. Role in Cl- secretion and evidence for two types of K+ channel.
The American journal of physiology 1990;258(6 Pt 1):L343-8.
1990: J D McCann; J Matsuda; M Garcia; G Kaczorowski; Michael J Welsh
Basolateral K+ channels in airway epithelia. I. Regulation by Ca2+ and block by charybdotoxin.
The American journal of physiology 1990;258(6 Pt 1):L334-42.
1990: K H Krause; Michael J Welsh
Voltage-dependent and Ca2(+)-activated ion channels in human neutrophils.
The Journal of clinical investigation 1990;85(2):491-8.
1990: J P Clancy; J D McCann; M Li; Michael J Welsh
Calcium-dependent regulation of airway epithelial chloride channels.
The American journal of physiology 1990;258(2 Pt 1):L25-32.
1990: J D McCann; Michael J Welsh
Regulation of Cl- and K+ channels in airway epithelium.
Annual review of physiology 1990;52():115-35.
1989: Michael J Welsh; M Li; J D McCann
Activation of normal and cystic fibrosis Cl- channels by voltage, temperature, and trypsin.
The Journal of clinical investigation 1989;84(6):2002-7.
1989: J D McCann; M Li; Michael J Welsh
Identification and regulation of whole-cell chloride currents in airway epithelium.
The Journal of general physiology 1989;94(6):1015-36.
1989: J D McCann; Ramesh C Bhalla; Michael J Welsh
Release of intracellular calcium by two different second messengers in airway epithelium.
The American journal of physiology 1989;257(2 Pt 1):L116-24.
1989: T Bainbridge; Ross D Feldman; Michael J Welsh
Adrenergic stimulation of inositol phosphate accumulation in tracheal epithelium.
Journal of applied physiology (Bethesda, Md. : 1985) 1989;66(1):504-8.
1989: Michael J Welsh; M Li; J D McCann; J P Clancy; Matthew Peter Anderson
Phosphorylation-dependent regulation of apical membrane chloride channels in normal and cystic fibrosis airway epithelium.
Annals of the New York Academy of Sciences 1989;574():44-51.
1988: Peter M Snyder; K H Krause; Michael J Welsh
Inositol trisphosphate isomers, but not inositol 1,3,4,5-tetrakisphosphate, induce calcium influx in Xenopus laevis oocytes.
The Journal of biological chemistry 1988;263(23):11048-51.
1988: M Li; J D McCann; C M Liedtke; Angus Nairn; Paul Greengard; Michael J Welsh
Cyclic AMP-dependent protein kinase opens chloride channels in normal but not cystic fibrosis airway epithelium.
Nature 1988;331(6154):358-60.
1987: Michael J Welsh; R B Fick
Cystic fibrosis.
The Journal of clinical investigation 1987;80(6):1523-6.
1987: Michael J Welsh
Effect of phorbol ester and calcium ionophore on chloride secretion in canine tracheal epithelium.
The American journal of physiology 1987;253(6 Pt 1):C828-34.
1987: Michael J Welsh
Electrolyte transport by airway epithelia.
Physiological reviews 1987;67(4):1143-84.