Michael Welsh

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Chemicals & Drugs

Disorders

Cystic Fibrosis

Anatomy

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Sun Yishan; Liu Lei; Ben-Shahar Yehuda; Jacobs Julie S; Eberl Daniel F; Welsh Michael J
TRPA channels distinguish gravity sensing from hearing in Johnston's organ.
Shah Alok S; Ben-Shahar Yehuda; Moninger Thomas O; Kline Joel N; Welsh Michael J
Motile cilia of human airway epithelia are chemosensory.
Zha Xiang-ming; Costa Vivian; Harding Anne Marie S; Reznikov Leah; Benson Christopher J; Welsh Michael J
ASIC2 subunits target acid-sensing ion channels to the synapse via an association with PSD-95.

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All Publications

2009: Sun Yishan; Liu Lei; Ben-Shahar Yehuda; Jacobs Julie S; Eberl Daniel F; Welsh Michael J
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: Shah Alok S; Ben-Shahar Yehuda; Moninger Thomas O; Kline Joel N; Welsh Michael J
Motile cilia of human airway epithelia are chemosensory.
Science (New York, N.Y.) 2009;325(5944):1131-4.
2009: Zha Xiang-ming; Costa Vivian; Harding Anne Marie S; Reznikov Leah; Benson Christopher J; Welsh Michael J
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: Zha Xiang-ming; Wang Runping; Collier Dan M; Snyder Peter M; Wemmie John A; Welsh Michael J
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: Flynn Andrea N; Itani Omar A; Moninger Thomas O; Welsh Michael J
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: Butler J E; Lager K M; Splichal I; Francis D; Kacskovics I; Sinkora M; Wertz N; Sun J; Zhao Y; Brown W R; DeWald R; Dierks S; Muyldermans S; Lunney J K; McCray P B; Rogers C S; Welsh M J; Navarro P; Klobasa F; Habe F; Ramsoondar J
The piglet as a model for B cell and immune system development.
Veterinary immunology and immunopathology 2009;128(1-3):147-70.
2009: Schnizler Mikael K; Schnizler Katrin; Zha Xiang-Ming; Hall Duane D; Wemmie John A; Hell Johannes W; Welsh Michael J
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: Dong Qian; Randak Christoph O; Welsh Michael J
A mutation in CFTR modifies the effects of the adenylate kinase inhibitor Ap5A on channel gating.
Biophysical journal 2008;95(11):5178-85.
2008: Rogers Christopher S; Stoltz David A; Meyerholz David K; Ostedgaard Lynda S; Rokhlina Tatiana; Taft Peter J; Rogan Mark P; Pezzulo Alejandro A; Karp Philip H; Itani Omar A; Kabel Amanda C; Wohlford-Lenane Christine L; Davis Greg J; Hanfland Robert A; Smith Tony L; Samuel Melissa; Wax David; Murphy Clifton N; Rieke August; Whitworth Kristin; Uc Aliye; Starner Timothy D; Brogden Kim A; Shilyansky Joel; McCray Paul B; Zabner Joseph; Prather Randall S; Welsh Michael J
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: Ziemann Adam E; Schnizler Mikael K; Albert Gregory W; Severson Meryl A; Howard Matthew A; Welsh Michael J; Wemmie John A
Seizure termination by acidosis depends on ASIC1a.
Nature neuroscience 2008;11(7):816-22.
2008: Sun Xingshen; Yan Ziying; Yi Yaling; Li Ziyi; Lei Diana; Rogers Christopher S; Chen Juan; Zhang Yulong; Welsh Michael J; Leno Gregory H; Engelhardt John F
Adeno-associated virus-targeted disruption of the CFTR gene in cloned ferrets.
The Journal of clinical investigation 2008;118(4):1578-83.
2008: Rogers Christopher S; Hao Yanhong; Rokhlina Tatiana; Samuel Melissa; Stoltz David A; Li Yuhong; Petroff Elena; Vermeer Daniel W; Kabel Amanda C; Yan Ziying; Spate Lee; Wax David; Murphy Clifton N; Rieke August; Whitworth Kristin; Linville Michael L; Korte Scott W; Engelhardt John F; Welsh Michael J; Prather Randall S
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: Shah Alok S; Farmen Sara L; Moninger Thomas O; Businga Thomas R; Andrews Michael P; Bugge Kevin; Searby Charles C; Nishimura Darryl; Brogden Kim A; Kline Joel N; Sheffield Val C; Welsh Michael J
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: Petroff Elena Yermolaieva; Price Margaret P; Snitsarev Vladislav; Gong Huiyu; Korovkina Victoria; Abboud Francois M; Welsh Michael J
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: Friese Manuel A; Craner Matthew J; Etzensperger Ruth; Vergo Sandra; Wemmie John A; Welsh Michael J; Vincent Angela; Fugger Lars
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: Randak Christoph O; Welsh Michael J
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: Liu Lei; Li Yuhong; Wang Runping; Yin Chong; Dong Qian; Hing Huey; Kim Changsoo; Welsh Michael J
Drosophila hygrosensation requires the TRP channels water witch and nanchung.
Nature 2007;450(7167):294-8.
2007: Ostedgaard Lynda S; Rogers Christopher S; Dong Qian; Randak Christoph O; Vermeer Daniel W; Rokhlina Tatiana; Karp Philip H; Welsh Michael J
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: Vermeer Paola D; McHugh Julia; Rokhlina Tatiana; Vermeer Daniel W; Zabner Joseph; Welsh Michael J
Vaccinia virus entry, exit, and interaction with differentiated human airway epithelia.
Journal of virology 2007;81(18):9891-9.
2007: Ben-Shahar Yehuda; Nannapaneni Kishore; Casavant Thomas L; Scheetz Todd E; Welsh Michael J
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: Zha Xiang-ming; Wemmie John A; Green Steven H; Welsh Michael J
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: Wemmie John A; Price Margaret P; Welsh Michael J
Acid-sensing ion channels: advances, questions and therapeutic opportunities.
Trends in neurosciences 2006;29(10):578-86.
2006: Vermeer Paola D; Panko Lacey; Welsh Michael J; Zabner Joseph
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: Farmen Sara L; Karp Philip H; Ng Philip; Palmer Donna J; Koehler David R; Hu Jim; Beaudet Arthur L; Zabner Joseph; Welsh Michael J
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: Randak Christoph O; Welsh Michael J
Adenylate kinase activity in ABC transporters.
The Journal of biological chemistry 2005;280(41):34385-8.
2005: Dajani Rana; Zhang Yulong; Taft Peter J; Travis Sue M; Starner Timothy D; Olsen Ansgar; Zabner Joseph; Welsh Michael J; Engelhardt John F
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: Ostedgaard Lynda S; Rokhlina Tatiana; Karp Philip H; Lashmit Philip; Afione Sandra; Schmidt Michael; Zabner Joseph; Stinski Mark F; Chiorini Jay A; Welsh Michael J
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: Randak Christoph O; Welsh Michael J
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: Berger Allan L; Randak Christoph O; Ostedgaard Lynda S; Karp Philip H; Vermeer Daniel W; Welsh Michael J
Curcumin stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.
The Journal of biological chemistry 2005;280(7):5221-6.
2005: Berger Allan L; Ikuma Mutsuhiro; Welsh Michael J
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: Page Amanda J; Brierley Stuart M; Martin Christopher M; Martinez-Salgado Carlos; Wemmie John A; Brennan Timothy J; Symonds Erin; Omari Taher; Lewin Gary R; Welsh Michael J; Blackshaw L Ashley
The ion channel ASIC1 contributes to visceral but not cutaneous mechanoreceptor function.
Gastroenterology 2004;127(6):1739-47.
2004: Hruska-Hageman Alesia M; Benson Christopher J; Leonard A Soren; Price Margaret P; Welsh Michael J
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: Xiong Zhi-Gang; Zhu Xiao-Man; Chu Xiang-Ping; Minami Manabu; Hey Jessica; Wei Wen-Li; MacDonald John F; Wemmie John A; Price Margaret P; Welsh Michael J; Simon Roger P
Neuroprotection in ischemia: blocking calcium-permeable acid-sensing ion channels.
Cell 2004;118(6):687-98.
2004: Yermolaieva Olena; Leonard A Soren; Schnizler Mikael K; Abboud Francois M; Welsh Michael J
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: Askwith Candice C; Wemmie John A; Price Margaret P; Rokhlina Tania; Welsh Michael J
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: Wemmie John A; Coryell Matthew W; Askwith Candice C; Lamani Ejvis; Leonard A Soren; Sigmund Curt D; Welsh Michael J
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: Scheetz Todd E; Zabner Joseph; Welsh Michael J; Coco Justin; Eyestone Mari de Fatima; Bonaldo Maria; Kucaba Tamara; Casavant Thomas L; Soares M Bento; McCray Paul B
Large-scale gene discovery in human airway epithelia reveals novel transcripts.
Physiological genomics 2004;17(1):69-77.
2004: Lee John H; Yi Su Min P; Anderson Mary E; Berger Kristi L; Welsh Michael J; Klingelhutz Aloysius J; Ozbun Michelle A
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: Randak Christoph; Welsh Michael J
An intrinsic adenylate kinase activity regulates gating of the ABC transporter CFTR.
Cell 2003;115(7):837-50.
2003: Sluka Kathleen A; Price Margaret P; Breese Nicole M; Stucky Cheryl L; Wemmie John A; Welsh Michael J
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: Ainsley Joshua A; Pettus Janette M; Bosenko Dmitry; Gerstein Clare E; Zinkevich Natalya; Anderson Michael G; Adams Christopher M; Welsh Michael J; Johnson Wayne A
Enhanced locomotion caused by loss of the Drosophila DEG/ENaC protein Pickpocket1.
Current biology : CB 2003;13(17):1557-63.
2003: Liu Lei; Leonard A Soren; Motto David G; Feller Margaret A; Price Margaret P; Johnson Wayne A; Welsh Michael J
Contribution of Drosophila DEG/ENaC genes to salt taste.
Neuron 2003;39(1):133-46.
2003: Wemmie John A; Askwith Candice C; Lamani Ejvis; Cassell Martin D; Freeman John H; Welsh Michael J
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: Xie Jinghui; Price Margaret P; Wemmie John A; Askwith Candice C; Welsh Michael J
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: Vermeer Paola D; Einwalter Lisa A; Moninger Thomas O; Rokhlina Tatiana; Kern Jeffrey A; Zabner Joseph; Welsh Michael J
Segregation of receptor and ligand regulates activation of epithelial growth factor receptor.
Nature 2003;422(6929):322-6.
2003: Liu Lei; Yermolaieva Olena; Johnson Wayne A; Abboud Francois M; Welsh Michael J
Identification and function of thermosensory neurons in Drosophila larvae.
Nature neuroscience 2003;6(3):267-73.
2003: Ostedgaard Lynda S; Randak Christoph; Rokhlina Tatiana; Karp Philip; Vermeer Daniel; Ashbourne Excoffon Katherine J; Welsh Michael J
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: Leonard A Soren; Yermolaieva Olena; Hruska-Hageman Alesia; Askwith Candice C; Price Margaret P; Wemmie John A; Welsh Michael J
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: Liu Lei; Johnson Wayne A; Welsh Michael J
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: Walters Robert W; Freimuth Paul; Moninger Thomas O; Ganske Ingrid; Zabner Joseph; Welsh Michael J
Adenovirus fiber disrupts CAR-mediated intercellular adhesion allowing virus escape.
Cell 2002;110(6):789-99.
2002: Xie Jinghui; Price Margaret P; Berger Allan L; Welsh Michael J
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: Singh Pradeep K; Parsek Matthew R; Greenberg E Peter; Welsh Michael J
A component of innate immunity prevents bacterial biofilm development.
Nature 2002;417(6888):552-5.
2002: Wemmie John A; Chen Jianguo; Askwith Candice C; Hruska-Hageman Alesia M; Price Margaret P; Nolan Brian C; Yoder Patrick G; Lamani Ejvis; Hoshi Toshinori; Freeman John H; Welsh Michael J
The acid-activated ion channel ASIC contributes to synaptic plasticity, learning, and memory.
Neuron 2002;34(3):463-77.
2002: Ostedgaard Lynda S; Zabner Joseph; Vermeer Daniel W; Rokhlina Tatiana; Karp Philip H; Stecenko Arlene A; Randak Christoph; Welsh Michael J
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: Benson Christopher J; Xie Jinghui; Wemmie John A; Price Margaret P; Henss Jillian M; Welsh Michael J; Snyder Peter M
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: Schutte Brian C; Mitros Joseph P; Bartlett Jennifer A; Walters Jesse D; Jia Hong Peng; Welsh Michael J; Casavant Thomas L; McCray Paul B
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: Hruska-Hageman Alesia M; Wemmie John A; Price Margaret P; Welsh Michael J
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: Berger Allan L; Ikuma Mutsuhiro; Hunt John F; Thomas Philip J; Welsh Michael J
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: Welsh Michael J; Price Margaret P; Xie Jinghui
Biochemical basis of touch perception: mechanosensory function of degenerin/epithelial Na+ channels.
The Journal of biological chemistry 2002;277(4):2369-72.
2002: Karp Philip H; Moninger Thomas O; Weber S Pary; Nesselhauf Tamara S; Launspach Janice L; Zabner Joseph; Welsh Michael J
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: Yi S M; Harson R E; Zabner J; Welsh M J
Lectin binding and endocytosis at the apical surface of human airway epithelia.
Gene therapy 2001;8(24):1826-32.
2001: Price M P; McIlwrath S L; Xie J; Cheng C; Qiao J; Tarr D E; Sluka K A; Brennan T J; Lewin G R; Welsh M J
The DRASIC cation channel contributes to the detection of cutaneous touch and acid stimuli in mice.
Neuron 2001;32(6):1071-83.
2001: Walters R W; van't Hof W; Yi S M; Schroth M K; Zabner J; Crystal R G; Welsh M J
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: Welsh M J; Smith J J
cAMP stimulation of HCO3- secretion across airway epithelia.
JOP : Journal of the pancreas 2001;2(4 Suppl):291-3.
2001: Yi S M; Lee J H; Graham S; Zabner J; Welsh M J
Adenovirus calcium phosphate coprecipitates enhance squamous cell carcinoma gene transfer.
The Laryngoscope 2001;111(7):1290-6.
2001: Prince L S; Karp P H; Moninger T O; Welsh M J
KGF alters gene expression in human airway epithelia: potential regulation of the inflammatory response.
Physiological genomics 2001;6(2):81-9.
2001: Drummond H A; Welsh M J; Abboud F M
ENaC subunits are molecular components of the arterial baroreceptor complex.
Annals of the New York Academy of Sciences 2001;940():42-7.
2001: Walters R W; Yi S M; Keshavjee S; Brown K E; Welsh M J; Chiorini J A; Zabner J
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: Askwith C C; Benson C J; Welsh M J; Snyder P M
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: Hennager D J; Ikuma M; Hoshi T; Welsh M J
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: Ostedgaard L S; Baldursson O; Welsh M J
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: Travis S M; Singh P K; Welsh M J
Antimicrobial peptides and proteins in the innate defense of the airway surface.
Current opinion in immunology 2001;13(1):89-95.
2001: Baldursson O; Ostedgaard L S; Rokhlina T; Cotten J F; Welsh M J
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: Drummond H A; Abboud F M; Welsh M J
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: Baldursson O; Berger H A; Welsh M J
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: Singh P K; Tack B F; McCray P B; Welsh M J
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: Price M P; Lewin G R; McIlwrath S L; Cheng C; Xie J; Heppenstall P A; Stucky C L; Mannsfeldt A G; Brennan T J; Drummond H A; Qiao J; Benson C J; Tarr D E; Hrstka R F; Yang B; Williamson R A; Welsh M J
The mammalian sodium channel BNC1 is required for normal touch sensation.
Nature 2000;407(6807):1007-11.
2000: Singh P K; Schaefer A L; Parsek M R; Moninger T O; Welsh M J; Greenberg E P
Quorum-sensing signals indicate that cystic fibrosis lungs are infected with bacterial biofilms.
Nature 2000;407(6805):762-4.
2000: Zabner J; Seiler M P; Launspach J L; Karp P H; Kearney W R; Look D C; Smith J J; Welsh M J
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: Berger A L; Welsh M J
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: Ikuma M; Welsh M J
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: Ostedgaard L S; Baldursson O; Vermeer D W; Welsh M J; Robertson A D
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: Travis S M; Anderson N N; Forsyth W R; Espiritu C; Conway B D; Greenberg E P; McCray P B; Lehrer R I; Welsh M J; Tack B F
Bactericidal activity of mammalian cathelicidin-derived peptides.
Infection and immunity 2000;68(5):2748-55.
2000: Askwith C C; Cheng C; Ikuma M; Benson C; Price M P; Welsh M J
Neuropeptide FF and FMRFamide potentiate acid-evoked currents from sensory neurons and proton-gated DEG/ENaC channels.
Neuron 2000;26(1):133-41.
2000: Drapkin P T; O'Riordan C R; Yi S M; Chiorini J A; Cardella J; Zabner J; Welsh M J
Targeting the urokinase plasminogen activator receptor enhances gene transfer to human airway epithelia.
The Journal of clinical investigation 2000;105(5):589-96.
2000: Walters R W; Duan D; Engelhardt J F; Welsh M J
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: Prince L S; Launspach J L; Geller D S; Lifton R P; Pratt J H; Zabner J; Welsh M J
Absence of amiloride-sensitive sodium absorption in the airway of an infant with pseudohypoaldosteronism.
The Journal of pediatrics 1999;135(6):786-9.
1999: Welsh M J
Gene transfer for cystic fibrosis.
The Journal of clinical investigation 1999;104(9):1165-6.
1999: Lee J H; Baker T J; Mahal L K; Zabner J; Bertozzi C R; Wiemer D F; Welsh M J
Engineering novel cell surface receptors for virus-mediated gene transfer.
The Journal of biological chemistry 1999;274(31):21878-84.
1999: McCray P B; Zabner J; Jia H P; Welsh M J; Thorne P S
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: Ostedgaard L S; Zeiher B; Welsh M J
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: Welsh M J; Zabner J
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: Prince L S; Welsh M J
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: Adams C M; Snyder P M; Welsh M J
Paradoxical stimulation of a DEG/ENaC channel by amiloride.
The Journal of biological chemistry 1999;274(22):15500-4.
1999: Travis S M; Conway B A; Zabner J; Smith J J; Anderson N N; Singh P K; Greenberg E P; Welsh M J
Activity of abundant antimicrobials of the human airway.
American journal of respiratory cell and molecular biology 1999;20(5):872-9.
1999: Lee J H; Welsh M J
Enhancement of calcium phosphate-mediated transfection by inclusion of adenovirus in coprecipitates.
Gene therapy 1999;6(4):676-82.
1999: Graham S M; Launspach J L; Welsh M J; Zabner J
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: Walters R W; Grunst T; Bergelson J M; Finberg R W; Welsh M J; Zabner J
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: Lee J H; Zabner J; Welsh M J
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: Adams C M; Price M P; Snyder P M; Welsh M J
Tetraethylammonium block of the BNC1 channel.
Biophysical journal 1999;76(3):1377-83.
1999: Chillon M; Bosch A; Zabner J; Law L; Armentano D; Welsh M J; Davidson B L
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: Cotten J F; Welsh M J
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: McDonald F J; Yang B; Hrstka R F; Drummond H A; Tarr D E; McCray P B; Stokes J B; Welsh M J; Williamson R A
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: Drummond H A; Price M P; Welsh M J; Abboud F M
A molecular component of the arterial baroreceptor mechanotransducer.
Neuron 1998;21(6):1435-41.
1998: Welsh M J; Robertson A D; Ostedgaard L S
Structural biology. The ABC of a versatile engine.
Nature 1998;396(6712):623-4.
1998: Singh P K; Jia H P; Wiles K; Hesselberth J; Liu L; Conway B A; Greenberg E P; Valore E V; Welsh M J; Ganz T; Tack B F; McCray P B
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: Prince L S; Welsh M J
Cell surface expression and biosynthesis of epithelial Na+ channels.
The Biochemical journal 1998;336 ( Pt 3)():705-10.
1998: Cotten J F; Welsh M J
Covalent modification of the nucleotide binding domains of cystic fibrosis transmembrane conductance regulator.
The Journal of biological chemistry 1998;273(48):31873-9.
1998: Adams C M; Snyder P M; Price M P; Welsh M J
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: Toyoda K; Ooboshi H; Chu Y; Fasbender A; Davidson B L; Welsh M J; Heistad D D
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: Hautala T; Grunst T; Fabrega A; Freimuth P; Welsh M J
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: Zabner J; Smith J J; Karp P H; Widdicombe J H; Welsh M J
Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro.
Molecular cell 1998;2(3):397-403.
1998: Cheng C; Prince L S; Snyder P M; Welsh M J
Assembly of the epithelial Na+ channel evaluated using sucrose gradient sedimentation analysis.
The Journal of biological chemistry 1998;273(35):22693-700.
1998: Chillón M; Lee J H; Fasbender A; Welsh M J
Adenovirus complexed with polyethylene glycol and cationic lipid is shielded from neutralizing antibodies in vitro.
Gene therapy 1998;5(7):995-1002.
1998: Hall R A; Ostedgaard L S; Premont R T; Blitzer J T; Rahman N; Welsh M J; Lefkowitz R J
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: Fasbender A; Lee J H; Walters R W; Moninger T O; Zabner J; Welsh M J
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: Berger H A; Travis S M; Welsh M J
Fluoride stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.
The American journal of physiology 1998;274(3 Pt 1):L305-12.
1998: Adams C M; Anderson M G; Motto D G; Price M P; Johnson W A; Welsh M J
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: Snyder P M; Cheng C; Prince L S; Rogers J C; Welsh M J
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: Fasbender A; Zabner J; Zeiher B G; Welsh M J
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: Ishihara H; Welsh M J
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: Adams C M; Snyder P M; Welsh M J
Interactions between subunits of the human epithelial sodium channel.
The Journal of biological chemistry 1997;272(43):27295-300.
1997: Cotten J F; Welsh M J
Covalent modification of the regulatory domain irreversibly stimulates cystic fibrosis transmembrane conductance regulator.
The Journal of biological chemistry 1997;272(41):25617-22.
1997: Travis S M; Berger H A; Welsh M J
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: Winter M C; Welsh M J
Stimulation of CFTR activity by its phosphorylated R domain.
Nature 1997;389(6648):294-6.
1997: Zabner J; Cheng S H; Meeker D; Launspach J; Balfour R; Perricone M A; Morris J E; Marshall J; Fasbender A; Smith A E; Welsh M J
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: Zabner J; Freimuth P; Puga A; Fabrega A; Welsh M J
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: Travis S M; Welsh M J
PP2C gamma: a human protein phosphatase with a unique acidic domain.
FEBS letters 1997;412(3):415-9.
1997: Fasbender A; Marshall J; Moninger T O; Grunst T; Cheng S; Welsh M J
Effect of co-lipids in enhancing cationic lipid-mediated gene transfer in vitro and in vivo.
Gene therapy 1997;4(7):716-25.
1997: Muhonen M G; Ooboshi H; Welsh M J; Davidson B L; Heistad D D
Gene transfer to cerebral blood vessels after subarachnoid hemorrhage.
Stroke; a journal of cerebral circulation 1997;28(4):822-8; discussion 828-9.
1997: Fasbender A; Zabner J; Chillón M; Moninger T O; Puga A P; Davidson B L; Welsh M J
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: Ostedgaard L S; Rich D P; DeBerg L G; Welsh M J
Association of domains within the cystic fibrosis transmembrane conductance regulator.
Biochemistry 1997;36(6):1287-94.
1996: Price M P; Ishihara H; Sheppard D N; Welsh M J
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: Zabner J; Zeiher B G; Friedman E; Welsh M J
Adenovirus-mediated gene transfer to ciliated airway epithelia requires prolonged incubation time.
Journal of virology 1996;70(10):6994-7003.
1996: Matsushita K; McCray P B; Sigmund R D; Welsh M J; Stokes J B
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: Cotten J F; Ostedgaard L S; Carson M R; Welsh M J
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: Sheppard D N; Travis S M; Ishihara H; Welsh M J
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: Zabner J; Wadsworth S C; Smith A E; Welsh M J
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: DeLisle S; Blondel O; Longo F J; Schnabel W E; Bell G I; Welsh M J
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: Price M P; Snyder P M; Welsh M J
Cloning and expression of a novel human brain Na+ channel.
The Journal of biological chemistry 1996;271(14):7879-82.
1996: Smith J J; Travis S M; Greenberg E P; Welsh M J
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.
Cell 1996;85(2):229-36.
1996: Teem J L; Carson M R; Welsh M J
Mutation of R555 in CFTR-delta F508 enhances function and partially corrects defective processing.
Receptors & channels 1996;4(1):63-72.
1995: Volk K A; Sigmund R D; Snyder P M; McDonald F J; Welsh M J; Stokes J B
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: Carson M R; Welsh M J
Structural and functional similarities between the nucleotide-binding domains of CFTR and GTP-binding proteins.
Biophysical journal 1995;69(6):2443-8.
1995: McDonald F J; Welsh M J
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: Snyder P M; Price M P; McDonald F J; Adams C M; Volk K A; Zeiher B G; Stokes J B; Welsh M J
Mechanism by which Liddle's syndrome mutations increase activity of a human epithelial Na+ channel.
Cell 1995;83(6):969-78.
1995: Zeiher B G; Eichwald E; Zabner J; Smith J J; Puga A P; McCray P B; Capecchi M R; Welsh M J; Thomas K R
A mouse model for the delta F508 allele of cystic fibrosis.
The Journal of clinical investigation 1995;96(4):2051-64.
1995: Carson M R; Winter M C; Travis S M; Welsh M J
Pyrophosphate stimulates wild-type and mutant cystic fibrosis transmembrane conductance regulator Cl- channels.
The Journal of biological chemistry 1995;270(35):20466-72.
1995: Zabner J; Fasbender A J; Moninger T; Poellinger K A; Welsh M J
Cellular and molecular barriers to gene transfer by a cationic lipid.
The Journal of biological chemistry 1995;270(32):18997-9007.
1995: Fasbender A J; Zabner J; Welsh M J
Optimization of cationic lipid-mediated gene transfer to airway epithelia.
The American journal of physiology 1995;269(1 Pt 1):L45-51.
1995: Ooboshi H; Welsh M J; Rios C D; Davidson B L; Heistad D D
Adenovirus-mediated gene transfer in vivo to cerebral blood vessels and perivascular tissue.
Circulation research 1995;77(1):7-13.
1995: DeLisle S; Mayr G W; Welsh M J
Inositol phosphate structural requisites for Ca2+ influx.
The American journal of physiology 1995;268(6 Pt 1):C1485-91.
1995: McCray P B; Armstrong K; Zabner J; Miller D W; Koretzky G A; Couture L; Robillard J E; Smith A E; Welsh M J
Adenoviral-mediated gene transfer to fetal pulmonary epithelia in vitro and in vivo.
The Journal of clinical investigation 1995;95(6):2620-32.
1995: McDonald F J; Price M P; Snyder P M; Welsh M J
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: Cheng S H; Fang S L; Zabner J; Marshall J; Piraino S; Schiavi S C; Jefferson D M; Welsh M J; Smith A E
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: Sheppard D N; Ostedgaard L S; Winter M C; Welsh M J
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: Welsh M J; Zabner J; Graham S M; Smith A E; Moscicki R; Wadsworth S
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: Carson M R; Travis S M; Welsh M J
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: Carson M R; Travis S M; Winter M C; Sheppard D N; Welsh M J
Phosphate stimulates CFTR Cl- channels.
Biophysical journal 1994;67(5):1867-75.
1994: Snyder P M; McDonald F J; Stokes J B; Welsh M J
Membrane topology of the amiloride-sensitive epithelial sodium channel.
The Journal of biological chemistry 1994;269(39):24379-83.
1994: Takahashi T; Matsushita K; Welsh M J; Stokes J B
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: McDonald F J; Snyder P M; McCray P B; Welsh M J
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: Winter M C; Sheppard D N; Carson M R; Welsh M J
Effect of ATP concentration on CFTR Cl- channels: a kinetic analysis of channel regulation.
Biophysical journal 1994;66(5):1398-403.
1994: Zabner J; Couture L A; Smith A E; Welsh M J
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: Sheppard D N; Carson M R; Ostedgaard L S; Denning G M; Welsh M J
Expression of cystic fibrosis transmembrane conductance regulator in a model epithelium.
The American journal of physiology 1994;266(4 Pt 1):L405-13.
1994: Smith J J; Karp P H; Welsh M J
Defective fluid transport by cystic fibrosis airway epithelia.
The Journal of clinical investigation 1994;93(3):1307-11.
1994: Sheppard D N; Ostedgaard L S; Rich D P; Welsh M J
The amino-terminal portion of CFTR forms a regulated Cl- channel.
Cell 1994;76(6):1091-8.
1994: DeLisle S; Radenberg T; Wintermantel M R; Tietz C; Parys J B; Pittet D; Welsh M J; Mayr G W
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: Welsh M J; Smith A E; Zabner J; Rich D P; Graham S M; Gregory R J; Pratt B M; Moscicki R A
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: Marshall J; Fang S; Ostedgaard L S; O'Riordan C R; Ferrara D; Amara J F; Hoppe H; Scheule R K; Welsh M J; Smith A E
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: Berger H A; Rich D P; Cheng S H; Travis S M; Saxena M; Smith A E; Welsh M J
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: Sheppard D N; Welsh M J
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: McCray P B; Bettencourt J D; Bastacky J; Denning G M; Welsh M J
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: Zabner J; Couture L A; Gregory R J; Graham S M; Smith A E; Welsh M J
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: Rich D P; Berger H A; Cheng S H; Travis S M; Saxena M; Smith A E; Welsh M J
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: Rich D P; Couture L A; Cardoza L M; Guiggio V M; Armentano D; Espino P C; Hehir K; Welsh M J; Smith A E; Gregory R J
Development and analysis of recombinant adenoviruses for gene therapy of cystic fibrosis.
Human gene therapy 1993;4(4):461-76.
1993: Travis S M; Carson M R; Ries D R; Welsh M J
Interaction of nucleotides with membrane-associated cystic fibrosis transmembrane conductance regulator.
The Journal of biological chemistry 1993;268(21):15336-9.
1993: Carson M R; Welsh M J
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: Welsh M J; Smith A E
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis.
Cell 1993;73(7):1251-4.
1993: Smith J J; Welsh M J
Fluid and electrolyte transport by cultured human airway epithelia.
The Journal of clinical investigation 1993;91(4):1590-7.
1993: Teem J L; Berger H A; Ostedgaard L S; Rich D P; Tsui L C; Welsh M J
Identification of revertants for the cystic fibrosis delta F508 mutation using STE6-CFTR chimeras in yeast.
Cell 1993;73(2):335-46.
1993: Sheppard D N; Rich D P; Ostedgaard L S; Gregory R J; Smith A E; Welsh M J
Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties.
Nature 1993;362(6416):160-4.
1993: Berger H A; Travis S M; Welsh M J
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: Welsh M J; Anderson M P
Regulation of the cystic fibrosis transmembrane conductance regulator chloride channel by MgATP.
Society of General Physiologists series 1993;48():119-27.
1993: Rich D P; Gregory R J; Cheng S H; Smith A E; Welsh M J
Effect of deletion mutations on the function of CFTR chloride channels.
Receptors & channels 1993;1(3):221-32.
1993: Welsh M J; Denning G M; Ostedgaard L S; Anderson M P
Dysfunction of CFTR bearing the delta F508 mutation.
Journal of cell science. Supplement 1993;17():235-9.
1992: Ostedgaard L S; Welsh M J
Partial purification of the cystic fibrosis transmembrane conductance regulator.
The Journal of biological chemistry 1992;267(36):26142-9.
1992: Sheppard D N; Welsh M J
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: Anderson M P; Welsh M J
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: Parys J B; Sernett S W; DeLisle S; Snyder P M; Welsh M J; Campbell K P
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: Denning G M; Anderson M P; Amara J F; Marshall J; Smith A E; Welsh M J
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive.
Nature 1992;358(6389):761-4.
1992: Denning G M; Ostedgaard L S; Welsh M J
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: Ostedgaard L S; Shasby D M; Welsh M J
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: Anderson M P; Sheppard D N; Berger H A; Welsh M J
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: DeLisle S; Pittet D; Potter B V; Lew P D; Welsh M J
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: Welsh M J; Anderson M P; Rich D P; Berger H A; Denning G M; Ostedgaard L S; Sheppard D N; Cheng S H; Gregory R J; Smith A E
Cystic fibrosis transmembrane conductance regulator: a chloride channel with novel regulation.
Neuron 1992;8(5):821-9.
1992: Tilly B C; Winter M C; Ostedgaard L S; O'Riordan C; Smith A E; Welsh M J
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: DeLisle S; Welsh M J
Inositol trisphosphate is required for the propagation of calcium waves in Xenopus oocytes.
The Journal of biological chemistry 1992;267(12):7963-6.
1992: Smith J J; Welsh M J
cAMP stimulates bicarbonate secretion across normal, but not cystic fibrosis airway epithelia.
The Journal of clinical investigation 1992;89(4):1148-53.
1992: Denning G M; Ostedgaard L S; Cheng S H; Smith A E; Welsh M J
Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia.
The Journal of clinical investigation 1992;89(1):339-49.
1991: Anderson M P; Berger H A; Rich D P; Gregory R J; Smith A E; Welsh M J
Nucleoside triphosphates are required to open the CFTR chloride channel.
Cell 1991;67(4):775-84.
1991: Berger H A; Anderson M P; Gregory R J; Thompson S; Howard P W; Maurer R A; Mulligan R; Smith A E; Welsh M J
Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channel.
The Journal of clinical investigation 1991;88(4):1422-31.
1991: Cheng S H; Rich D P; Marshall J; Gregory R J; Welsh M J; Smith A E
Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel.
Cell 1991;66(5):1027-36.
1991: Gregory R J; Rich D P; Cheng S H; Souza D W; Paul S; Manavalan P; Anderson M P; Welsh M J; Smith A E
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: Rich D P; Gregory R J; Anderson M P; Manavalan P; Smith A E; Welsh M J
Effect of deleting the R domain on CFTR-generated chloride channels.
Science (New York, N.Y.) 1991;253(5016):205-7.
1991: Anderson M P; Gregory R J; Thompson S; Souza D W; Paul S; Mulligan R C; Smith A E; Welsh M J
Demonstration that CFTR is a chloride channel by alteration of its anion selectivity.
Science (New York, N.Y.) 1991;253(5016):202-5.
1991: Anderson M P; Welsh M J
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: Denning G M; Welsh M J
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: Berger H A; Welsh M J
Electrolyte transport in the lungs.
Hospital practice (Office ed.) 1991;26(3):53-9.
1991: Anderson M P; Rich D P; Gregory R J; Smith A E; Welsh M J
Generation of cAMP-activated chloride currents by expression of CFTR.
Science (New York, N.Y.) 1991;251(4994):679-82.
1990: Feldman R D; Brotherton A; Welsh M J
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: Jefferson D M; Valentich J D; Marini F C; Grubman S A; Iannuzzi M C; Dorkin H L; Li M; Klinger K W; Welsh M J
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: Clancy J P; McCann J D; Welsh M J
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: Gregory R J; Cheng S H; Rich D P; Marshall J; Paul S; Hehir K; Ostedgaard L; Klinger K W; Welsh M J; Smith A E
Expression and characterization of the cystic fibrosis transmembrane conductance regulator.
Nature 1990;347(6291):382-6.
1990: Rich D P; Anderson M P; Gregory R J; Cheng S H; Paul S; Jefferson D M; McCann J D; Klinger K W; Smith A E; Welsh M J
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: Anderson M P; Welsh M J
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: Li M; McCann J D; Welsh M J
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: DeLisle S; Krause K H; Denning G; Potter B V; Welsh M J
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: Anderson M P; Welsh M J
Isoproterenol, cAMP, and bradykinin stimulate diacylglycerol production in airway epithelium.
The American journal of physiology 1990;258(6 Pt 1):L294-300.
1990: Smith J J; McCann J D; Welsh M J
Bradykinin stimulates airway epithelial Cl- secretion via two second messenger pathways.
The American journal of physiology 1990;258(6 Pt 1):L369-77.
1990: McCann J D; Welsh M J
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: McCann J D; Matsuda J; Garcia M; Kaczorowski G; Welsh M J
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: Krause K H; Welsh M J
Voltage-dependent and Ca2(+)-activated ion channels in human neutrophils.
The Journal of clinical investigation 1990;85(2):491-8.
1990: Clancy J P; McCann J D; Li M; Welsh M J
Calcium-dependent regulation of airway epithelial chloride channels.
The American journal of physiology 1990;258(2 Pt 1):L25-32.
1990: McCann J D; Welsh M J
Regulation of Cl- and K+ channels in airway epithelium.
Annual review of physiology 1990;52():115-35.
1989: Welsh M J; Li M; McCann J D
Activation of normal and cystic fibrosis Cl- channels by voltage, temperature, and trypsin.
The Journal of clinical investigation 1989;84(6):2002-7.
1989: McCann J D; Li M; Welsh M J
Identification and regulation of whole-cell chloride currents in airway epithelium.
The Journal of general physiology 1989;94(6):1015-36.
1989: McCann J D; Bhalla R C; Welsh M J
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: Bainbridge T; Feldman R D; Welsh M J
Adrenergic stimulation of inositol phosphate accumulation in tracheal epithelium.
Journal of applied physiology (Bethesda, Md. : 1985) 1989;66(1):504-8.
1989: Welsh M J; Li M; McCann J D; Clancy J P; Anderson M P
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: Snyder P M; Krause K H; Welsh M J
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: Li M; McCann J D; Liedtke C M; Nairn A C; Greengard P; Welsh M J
Cyclic AMP-dependent protein kinase opens chloride channels in normal but not cystic fibrosis airway epithelium.
Nature 1988;331(6154):358-60.
1987: Welsh M J; Fick R B
Cystic fibrosis.
The Journal of clinical investigation 1987;80(6):1523-6.
1987: Welsh M J
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: Welsh M J
Electrolyte transport by airway epithelia.
Physiological reviews 1987;67(4):1143-84.

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