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David Borchelt

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Tatiana Melnikova; Brenda D Moore; Ashleigh Price; Alena V Savonenko; Guilian Xu; Kevin M Felsenstein; Susan Fromholt; Todd E Golde; HyunSu Kim; Deidre Lee; David R Borchelt
Reversible pathologic and cognitive phenotypes in an inducible model of Alzheimer-amyloidosis.
Mercedes Prudencio; Joan S Valentine; Julian P Whitelegge; Hilda H Brown; Herman Lelie; David R Borchelt
A novel variant of human superoxide dismutase 1 harboring amyotrophic lateral sclerosis-associated and experimental mutations in metal-binding residues and free cysteines lacks toxicity in vivo.
Andrew T N Tebbenkamp; Hilda H Brown; Keith W Crosby; Todd E Golde; Zoe B Siemienski; David R Borchelt
Analysis of proteolytic processes and enzymatic activities in the generation of huntingtin n-terminal fragments in an HEK293 cell model.

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All Publications

2013: Tatiana Melnikova; Brenda D Moore; Ashleigh Price; Alena V Savonenko; Guilian Xu; Kevin M Felsenstein; Susan Fromholt; Todd E Golde; HyunSu Kim; Deidre Lee; David R Borchelt
Reversible pathologic and cognitive phenotypes in an inducible model of Alzheimer-amyloidosis.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2013;33(9):3765-79.
2012: Mercedes Prudencio; Joan S Valentine; Julian P Whitelegge; Hilda H Brown; Herman Lelie; David R Borchelt
A novel variant of human superoxide dismutase 1 harboring amyotrophic lateral sclerosis-associated and experimental mutations in metal-binding residues and free cysteines lacks toxicity in vivo.
Journal of neurochemistry 2012;121(3):475-85.
2012: Andrew T N Tebbenkamp; Hilda H Brown; Keith W Crosby; Todd E Golde; Zoe B Siemienski; David R Borchelt
Analysis of proteolytic processes and enzymatic activities in the generation of huntingtin n-terminal fragments in an HEK293 cell model.
PloS one 2012;7(12):e50750.
2012: Guilian Xu; Firas Kobeissy; Hilda Brown; Mark S Gold; Firas Kobiessy; Scott H McClung; Stanley M Stevens; David R Borchelt
Identification of proteins sensitive to thermal stress in human neuroblastoma and glioma cell lines.
PloS one 2012;7(11):e49021.
2012: Brittany L T Roberts; Hilda H Brown; Kinaree Patel; David R Borchelt
Role of disulfide cross-linking of mutant SOD1 in the formation of inclusion-body-like structures.
PloS one 2012;7(10):e47838.
2012: eMalick G Njie; Malisa Sarntinoranont; Susan L Semple-Rowland; Dennis A Steindler; Wolfgang J Streit; Tong Zheng; Garrett W Astary; Cameron Green; Svetlana Kantorovich; David R Borchelt
A preclinical assessment of neural stem cells as delivery vehicles for anti-amyloid therapeutics.
PloS one 2012;7(4):e34097.
2012: Emalick G Njie; Frank R M Stassen; Harry W M Steinbusch; Erik J H Boelen; David R Borchelt; Wolfgang J Streit
Ex vivo cultures of microglia from young and aged rodent brain reveal age-related changes in microglial function.
Neurobiology of aging 2012;33(1):195.e1-12.
2011: K Amy Chen; Pedro E Cruz; Derek J Lanuto; Terence R Flotte; David R Borchelt; Arun Srivastava; Jianyi Zhang; Dennis A Steindler; Tong Zheng
Cellular fusion for gene delivery to SCA1 affected Purkinje neurons.
Molecular and cellular neurosciences 2011;47(1):61-70.
2011: Herman L Lelie; A Liba; Megan W Bourassa; Madhuri Chattopadhyay; Pik K Chan; Edith Butler Gralla; Lisa M Miller; David R Borchelt; Joan Selverstone Valentine; Julian P Whitelegge
Copper and zinc metallation status of copper-zinc superoxide dismutase from amyotrophic lateral sclerosis transgenic mice.
The Journal of biological chemistry 2011;286(4):2795-806.
2011: Ying Liu; Michael K Lee; Maria M James; Donald L Price; David R Borchelt; Juan C Troncoso; ESTHER OH
Passive (amyloid-β) immunotherapy attenuates monoaminergic axonal degeneration in the AβPPswe/PS1dE9 mice.
Journal of Alzheimer's disease : JAD 2011;23(2):271-9.
2010: Celeste M Karch; David R Borchelt
Aggregation modulating elements in mutant human superoxide dismutase 1.
Archives of biochemistry and biophysics 2010;503(2):175-82.
2010: Wanli Smith; Zhaohui Liu; Yideng Liang; Naoki Masuda; Debbie A Swing; Nancy A Jenkins; Neal G Copeland; Juan C Troncoso; Mikhail V Pletnikov; Ted M Dawson; Lee J Martin; Timothy H Moran; Michael K Lee; David R Borchelt; Christopher A Ross
Synphilin-1 attenuates neuronal degeneration in the A53T alpha-synuclein transgenic mouse model.
Human molecular genetics 2010;19(11):2087-98.
2010: Celeste M Karch; David R Borchelt
An examination of alpha B-crystallin as a modifier of SOD1 aggregate pathology and toxicity in models of familial amyotrophic lateral sclerosis.
Journal of neurochemistry 2010;113(5):1092-100.
2010: Alexandra M Klevytska; Andrew T N Tebbenkamp; Alena V Savonenko; David R Borchelt
Partial depletion of CREB-binding protein reduces life expectancy in a mouse model of Huntington disease.
Journal of neuropathology and experimental neurology 2010;69(4):396-404.
2010: Andrew T N Tebbenkamp; David R Borchelt
Analysis of chaperone mRNA expression in the adult mouse brain by meta analysis of the Allen Brain Atlas.
PloS one 2010;5(10):e13675.
2009: Sai V Seetharaman; Mercedes Prudencio; Celeste Karch; Stephen P Holloway; David R Borchelt; Peter Hart
Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis.
Experimental biology and medicine (Maywood, N.J.) 2009;234(10):1140-54.
2009: Mercedes Prudencio; Peter Hart; David R Borchelt; Peter M Andersen
Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease.
Human molecular genetics 2009;18(17):3217-26.
2009: ESTHER OH; Alena V Savonenko; Julie F King; Stina M Fangmark Tucker; Gay Rudow; Guilian Xu; David R Borchelt; Juan C Troncoso
Amyloid precursor protein increases cortical neuron size in transgenic mice.
Neurobiology of aging 2009;30(8):1238-44.
2009: Celeste M Karch; Mercedes Prudencio; Duane D Winkler; Peter Hart; David R Borchelt
Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS.
Proceedings of the National Academy of Sciences of the United States of America 2009;106(19):7774-9.
2009: Duane D Winkler; Jonathan P Schuermann; Xiaohang Cao; Stephen P Holloway; David R Borchelt; Mark C Carroll; Jody B Proescher; Valeria Cizewski Culotta; Peter Hart
Structural and biophysical properties of the pathogenic SOD1 variant H46R/H48Q.
Biochemistry 2009;48(15):3436-47.
2009: Mercedes Prudencio; Armando Durazo; Julian P Whitelegge; David R Borchelt
Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme.
Journal of neurochemistry 2009;108(4):1009-18.
2009: Andrew T N Tebbenkamp; David R Borchelt
Protein aggregate characterization in models of neurodegenerative disease.
Methods in molecular biology (Clifton, N.J.) 2009;566():85-91.
2008: Ying Liu; Mi-Jeong Yoo; Alena V Savonenko; Wanda Stirling; Donald L Price; David R Borchelt; Laura Mamounas; W Ernest Lyons; Mary E Blue; Michael K Lee
Amyloid pathology is associated with progressive monoaminergic neurodegeneration in a transgenic mouse model of Alzheimer's disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2008;28(51):13805-14.
2008: Florian Muller; Yuhong Liu; Amanda Jernigan; David R Borchelt; Arlan G Richardson; Holly Van Remmen
MnSOD deficiency has a differential effect on disease progression in two different ALS mutant mouse models.
Muscle & nerve 2008;38(3):1173-83.
2008: Celeste M Karch; David R Borchelt
A limited role for disulfide cross-linking in the aggregation of mutant SOD1 linked to familial amyotrophic lateral sclerosis.
The Journal of biological chemistry 2008;283(20):13528-37.
2008: Jiou Wang; Elizabeth Martin; Victoria Gonzales; David R Borchelt; Michael K Lee
Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases.
Neurobiology of aging 2008;29(4):586-97.
2008: Bryan Francis Shaw; Herman L Lelie; Armando Durazo; Aram M Nersissian; Guilian Xu; Pik K Chan; Edith Butler Gralla; Ashutosh Tiwari; Lawrence J Hayward; David R Borchelt; Joan Selverstone Valentine; Julian P Whitelegge
Detergent-insoluble aggregates associated with amyotrophic lateral sclerosis in transgenic mice contain primarily full-length, unmodified superoxide dismutase-1.
The Journal of biological chemistry 2008;283(13):8340-50.
2008: Guilian Xu; Celeste Karch; Ning Li; Nianwei Lin; David Fromholt; Victoria Gonzales; David R Borchelt
Receptor-associated protein (RAP) plays a central role in modulating Abeta deposition in APP/PS1 transgenic mice.
PloS one 2008;3(9):e3159.
2008: Yueming Chang; Qiongman Kong; Xiu Shan; Guilian Tian; Hristelina Ilieva; Don W Cleveland; Jeffrey rothstein; David R Borchelt; Philip C Wong; Chien-liang Glenn Lin
Messenger RNA oxidation occurs early in disease pathogenesis and promotes motor neuron degeneration in ALS.
PloS one 2008;3(8):e2849.
2008: Stina M Fangmark Tucker; David R Borchelt; Juan C Troncoso
Limited clearance of pre-existing amyloid plaques after intracerebral injection of Abeta antibodies in two mouse models of Alzheimer disease.
Journal of neuropathology and experimental neurology 2008;67(1):30-40.
2007: TAMARA RATOVITSKI; Masayuki Nakamura; James D'Ambola; Ekaterine Chighladze; Yideng Liang; Wenfei Wang; Graham Kyrenia Rona; Michael R Hayden; David R Borchelt; RICKY HIRSCHHORN; Christopher A Ross
N-terminal proteolysis of full-length mutant huntingtin in an inducible PC12 cell model of Huntington's disease.
Cell cycle (Georgetown, Tex.) 2007;6(23):2970-81.
2007: Joanna Jankowsky; Linda Younkin; Victoria Gonzales; Daniel J Fadale; Hilda H Slunt; Henry A Lester; Steven G Younkin; David R Borchelt
Rodent A beta modulates the solubility and distribution of amyloid deposits in transgenic mice.
The Journal of biological chemistry 2007;282(31):22707-20.
2007: laure verret; Joanna Jankowsky; Guilian Xu; David R Borchelt; Claire Rampon
Alzheimer's-type amyloidosis in transgenic mice impairs survival of newborn neurons derived from adult hippocampal neurogenesis.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2007;27(25):6771-80.
2007: Gabriele Schilling; Alexandra Klevytska; Andrew T N Tebbenkamp; Katrin Juenemann; Jillian Cooper; Victoria Gonzales; Hilda H Slunt; Michelle Poirer; Christopher A Ross; David R Borchelt
Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models.
Journal of neuropathology and experimental neurology 2007;66(4):313-20.
2007: Jiou Wang; Amy Caruano-Yzermans; Angela Rodriguez; Jonathan P Scheurmann; Hilda H Slunt; Xiaohang Cao; Jonathan D Gitlin; Peter Hart; David R Borchelt
Disease-associated mutations at copper ligand histidine residues of superoxide dismutase 1 diminish the binding of copper and compromise dimer stability.
The Journal of biological chemistry 2007;282(1):345-52.
2007: Daniel Krewski; Robert A Yokel; Evert Nieboer; David R Borchelt; Joshua Cohen; Jean Harry; Sam Kacew; Joan Lindsay; Amal M Mahfouz; Virginie Rondeau
Human health risk assessment for aluminium, aluminium oxide, and aluminium hydroxide.
Journal of toxicology and environmental health. Part B, Critical reviews 2007;10 Suppl 1():1-269.
2007: Devin S Gary; Abigail Davidson; Olivier Milhavet; Hilda H Slunt; David R Borchelt
Investigation of RNA interference to suppress expression of full-length and fragment human huntingtin.
Neuromolecular medicine 2007;9(2):145-55.
2006: David R Borchelt
Amyotrophic lateral sclerosis--are microglia killing motor neurons?
The New England journal of medicine 2006;355(15):1611-3.
2006: Eduardo Zamora; Alessandra Handisurya; Saeed Shafti-Keramat; David R Borchelt; Gay Rudow; Katherine Conant; Christopher Cox; Juan C Troncoso; Reinhard Kirnbauer
Papillomavirus-like particles are an effective platform for amyloid-beta immunization in rabbits and transgenic mice.
Journal of immunology (Baltimore, Md. : 1950) 2006;177(4):2662-70.
2006: Jiou Wang; Guilian Xu; David R Borchelt
Mapping superoxide dismutase 1 domains of non-native interaction: roles of intra- and intermolecular disulfide bonding in aggregation.
Journal of neurochemistry 2006;96(5):1277-88.
2006: Yuji Tanaka; Shuichi Igarashi; Masayuki Nakamura; Juliette Gafni; Cameron Torcassi; Gabriele Schilling; Danielle Crippen; Jonathan D Wood; Akira Sawa; Nancy A Jenkins; Neal G Copeland; David R Borchelt; Christopher A Ross; Lisa M Ellerby
Progressive phenotype and nuclear accumulation of an amino-terminal cleavage fragment in a transgenic mouse model with inducible expression of full-length mutant huntingtin.
Neurobiology of disease 2006;21(2):381-91.
2005: Fiona M Laird; Huaibin Cai; Alena V Savonenko; Mohamed H Farah; Kaiwen He; Tatiana Melnikova; Hongjin Wen; Hsueh-Cheng Chiang; Guilian Xu; Vassilis E Koliatsos; David R Borchelt; Donald L Price; Hey-Kyoung Lee; Philip C Wong
BACE1, a major determinant of selective vulnerability of the brain to amyloid-beta amyloidogenesis, is essential for cognitive, emotional, and synaptic functions.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2005;25(50):11693-709.
2005: Joanna Jankowsky; Hilda H Slunt; Victoria Gonzales; Alena V Savonenko; Jason C Wen; Nancy A Jenkins; Neal G Copeland; Linda Younkin; Henry A Lester; Steven G Younkin; David R Borchelt
Persistent amyloidosis following suppression of Abeta production in a transgenic model of Alzheimer disease.
PLoS medicine 2005;2(12):e355.
2005: Jiou Wang; Guilian Xu; Hilda H Slunt; Victoria Gonzales; Michael Coonfield; David Fromholt; Neal G Copeland; Nancy A Jenkins; David R Borchelt
Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA.
Neurobiology of disease 2005;20(3):943-52.
2005: Bruce G Jenkins; Ole A Andreassen; Alpaslan Dedeoglu; Blair R Leavitt; Michael R Hayden; David R Borchelt; Christopher A Ross; Robert J Ferrante; M Flint Beal
Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease.
Journal of neurochemistry 2005;95(2):553-62.
2005: Jiou Wang; Guilian Xu; Hong Li; Victoria Gonzales; David Fromholt; Celeste Karch; Neal G Copeland; Nancy A Jenkins; David R Borchelt
Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: alphaB-crystallin modulates aggregation.
Human molecular genetics 2005;14(16):2335-47.
2005: Joanna Jankowsky; Tatiana Melnikova; Daniel J Fadale; Guilian Xu; Hilda H Slunt; Victoria Gonzales; Linda Younkin; Steven G Younkin; David R Borchelt; Alena V Savonenko
Environmental enrichment mitigates cognitive deficits in a mouse model of Alzheimer's disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2005;25(21):5217-24.
2005: Alena V Savonenko; Guilian Xu; Tatiana Melnikova; Johanna L Morton; Victoria Gonzales; Molly P F Wong; Donald L Price; Fai Tang; Alicja L Markowska; David R Borchelt
Episodic-like memory deficits in the APPswe/PS1dE9 mouse model of Alzheimer's disease: relationships to beta-amyloid deposition and neurotransmitter abnormalities.
Neurobiology of disease 2005;18(3):602-17.
2004: Joanna Jankowsky; Hilda H Slunt; Victoria Gonzales; Nancy A Jenkins; Neal G Copeland; David R Borchelt
APP processing and amyloid deposition in mice haplo-insufficient for presenilin 1.
Neurobiology of aging 2004;25(7):885-92.
2004: Gabriele Schilling; Alena V Savonenko; Alexandra Klevytska; Johanna L Morton; Stina M Tucker; Michelle A Poirier; Alexa Gale; Ning Chan; Victoria Gonzales; Hilda H Slunt; Michael Coonfield; Nancy A Jenkins; Neal G Copeland; Christopher A Ross; David R Borchelt
Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice.
Human molecular genetics 2004;13(15):1599-610.
2004: Gabriele Schilling; Alena V Savonenko; Michael Coonfield; Johanna L Morton; Esther Vorovich; Alexa Gale; Christopher Neslon; Ning Chan; Michelle Eaton; David Fromholt; Christopher A Ross; David R Borchelt
Environmental, pharmacological, and genetic modulation of the HD phenotype in transgenic mice.
Experimental neurology 2004;187(1):137-49.
2004: Joanna Jankowsky; Daniel J Fadale; Jeffrey Anderson; Guilian Xu; Victoria Gonzales; Nancy A Jenkins; Neal G Copeland; Michael K Lee; Linda Younkin; Steven L Wagner; Steven G Younkin; David R Borchelt
Mutant presenilins specifically elevate the levels of the 42 residue beta-amyloid peptide in vivo: evidence for augmentation of a 42-specific gamma secretase.
Human molecular genetics 2004;13(2):159-70.
2003: Joanna Jankowsky; Guilian Xu; David Fromholt; Victoria Gonzales; David R Borchelt
Environmental enrichment exacerbates amyloid plaque formation in a transgenic mouse model of Alzheimer disease.
Journal of neuropathology and experimental neurology 2003;62(12):1220-7.
2003: Jiou Wang; Hilda H Slunt; Victoria Gonzales; David Fromholt; Michael Coonfield; Neal G Copeland; Nancy A Jenkins; David R Borchelt
Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature.
Human molecular genetics 2003;12(21):2753-64.
2003: Jin G Sheng; Susan H Bora; Guilian Xu; David R Borchelt; Donald L Price; Vassilis E Koliatsos
Lipopolysaccharide-induced-neuroinflammation increases intracellular accumulation of amyloid precursor protein and amyloid beta peptide in APPswe transgenic mice.
Neurobiology of disease 2003;14(1):133-45.
2003: Alena V Savonenko; Guilian Xu; Donald L Price; David R Borchelt; Alicja L Markowska
Normal cognitive behavior in two distinct congenic lines of transgenic mice hyperexpressing mutant APP SWE.
Neurobiology of disease 2003;12(3):194-211.
2003: Flavio Kamenetz; Taisuke Tomita; Helen Hsieh; Guy Seabrook; David R Borchelt; Takeshi Iwatsubo; Sangram S Sisodia; Roberto Malinow
APP processing and synaptic function.
Neuron 2003;37(6):925-37.
2002: John E Kratz; duncan stearns; David L Huso; Hilda H Slunt; Donald L Price; David R Borchelt; Charles G Eberhart
Expression of stabilized beta-catenin in differentiated neurons of transgenic mice does not result in tumor formation.
BMC cancer 2002;2():33.
2002: Joanna Jankowsky; Alena V Savonenko; Gabriele Schilling; Jiou Wang; Guilian Xu; David R Borchelt
Transgenic mouse models of neurodegenerative disease: opportunities for therapeutic development.
Current neurology and neuroscience reports 2002;2(5):457-64.
2002: Ruth Luthi-Carter; Andrew D Strand; Sarah A Hanson; Charles Kooperberg; Gabriele Schilling; Albert R La Spada; Diane E Merry; Anne B Young; Christopher A Ross; David R Borchelt; James M Olson
Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects.
Human molecular genetics 2002;11(17):1927-37.
2002: Guilian Xu; Victoria Gonzales; David R Borchelt
Abeta deposition does not cause the aggregation of endogenous tau in transgenic mice.
Alzheimer disease and associated disorders 2002;16(3):196-201.
2002: Guilian Xu; Victoria Gonzales; David R Borchelt
Rapid detection of protein aggregates in the brains of Alzheimer patients and transgenic mouse models of amyloidosis.
Alzheimer disease and associated disorders 2002;16(3):191-5.
2002: Jiou Wang; Guilian Xu; Victoria Gonzales; Michael Coonfield; David Fromholt; Neal G Copeland; Nancy A Jenkins; David R Borchelt
Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site.
Neurobiology of disease 2002;10(2):128-38.
2002: Philip C Wong; Huaibin Cai; David R Borchelt; Donald L Price
Genetically engineered mouse models of neurodegenerative diseases.
Nature neuroscience 2002;5(7):633-9.
2002: Steven Estus; David R Borchelt; Mark S Kindy; Robert Vassar
Abeta deposition is essential to AD neuropathology.
Journal of Alzheimer's disease : JAD 2002;4(3):133-8.
2002: Zhongmin Xiang; Lap Ho; Jennifer Valdellon; David R Borchelt; Kevin Kelley; Lauren Spielman; Paul S Aisen; Giulio Maria Pasinetti
Cyclooxygenase (COX)-2 and cell cycle activity in a transgenic mouse model of Alzheimer's disease neuropathology.
Neurobiology of aging 2002;23(3):327-34.
2002: Vanessa C Wheeler; Claire-Anne Gutekunst; Vladimir Vrbanac; Lori-Anne Lebel; Gabriele Schilling; Steven M Hersch; Robert M Friedlander; James Gusella; Jean-Paul Vonsattel; David R Borchelt; Marcy E MacDonald
Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice.
Human molecular genetics 2002;11(6):633-40.
2002: Jiou Wang; Guilian Xu; David R Borchelt
High molecular weight complexes of mutant superoxide dismutase 1: age-dependent and tissue-specific accumulation.
Neurobiology of disease 2002;9(2):139-48.
2002: David R Borchelt; Michael K Lee; Victoria Gonzales; Hilda H Slunt; TAMARA RATOVITSKI; Nancy A Jenkins; Neal G Copeland; Donald L Price; Sangram S Sisodia
Accumulation of proteolytic fragments of mutant presenilin 1 and accelerated amyloid deposition are co-regulated in transgenic mice.
Neurobiology of aging 2002;23(2):171-7.
2001: Gabriele Schilling; Michael Coonfield; Christopher A Ross; David R Borchelt
Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model.
Neuroscience letters 2001;315(3):149-53.
2001: Anne K Vehmas; David R Borchelt; Donald L Price; D McCarthy; MARSHA WILLS-KARP; M J Peper; Gay Rudow; J Luyinbazi; L T Siew; Juan C Troncoso
beta-Amyloid peptide vaccination results in marked changes in serum and brain Abeta levels in APPswe/PS1DeltaE9 mice, as detected by SELDI-TOF-based ProteinChip technology.
DNA and cell biology 2001;20(11):713-21.
2001: Christian Lesuisse; Guilian Xu; J Anderson; M Wong; Joanna Jankowsky; G Holtz; V Gonzalez; Philip C Wong; Donald L Price; F Tang; S Wagner; David R Borchelt
Hyper-expression of human apolipoprotein E4 in astroglia and neurons does not enhance amyloid deposition in transgenic mice.
Human molecular genetics 2001;10(22):2525-37.
2001: Philip C Wong; Huaibin Cai; David R Borchelt; Donald L Price
Genetically engineered models relevant to neurodegenerative disorders: their value for understanding disease mechanisms and designing/testing experimental therapeutics.
Journal of molecular neuroscience : MN 2001;17(2):233-57.
2001: Ole A Andreassen; Robert J Ferrante; H M Huang; Alpaslan Dedeoglu; Lily Park; Kimberly L Ferrante; J Kwon; David R Borchelt; Christopher A Ross; G E Gibson; M Flint Beal
Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease.
Annals of neurology 2001;50(1):112-7.
2001: Ole A Andreassen; Alpaslan Dedeoglu; Robert J Ferrante; Bruce G Jenkins; Kimberly L Ferrante; M Thomas; Avi Friedlich; Susan E Browne; Gabriele Schilling; David R Borchelt; Steven M Hersch; Christopher A Ross; M Flint Beal
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease.
Neurobiology of disease 2001;8(3):479-91.
2001: Gabriele Schilling; Hyder A Jinnah; Victoria Gonzales; Michael Coonfield; Y Kim; Jonathan D Wood; Donald L Price; Xiao-Jiang Li; Nancy Jenkins; Neal G Copeland; Timothy H Moran; Christopher A Ross; David R Borchelt
Distinct behavioral and neuropathological abnormalities in transgenic mouse models of HD and DRPLA.
Neurobiology of disease 2001;8(3):405-18.
2001: Joanna Jankowsky; Hilda H Slunt; TAMARA RATOVITSKI; Nancy A Jenkins; Neal G Copeland; David R Borchelt
Co-expression of multiple transgenes in mouse CNS: a comparison of strategies.
Biomolecular engineering 2001;17(6):157-65.
2001: Huaibin Cai; Y Wang; D McCarthy; Hongjin Wen; David R Borchelt; Donald L Price; Philip C Wong
BACE1 is the major beta-secretase for generation of Abeta peptides by neurons.
Nature neuroscience 2001;4(3):233-4.
2000: Masaaki Takahashi; Sylvain Dore; Christopher D Ferris; Taisuke Tomita; Akira Sawa; H Wolosker; David R Borchelt; Takeshi Iwatsubo; Seong-Hun Kim; Gopal Thinakaran; Sangram S Sisodia; Solomon H Snyder
Amyloid precursor proteins inhibit heme oxygenase activity and augment neurotoxicity in Alzheimer's disease.
Neuron 2000;28(2):461-73.
2000: Ruth Luthi-Carter; Andrew D Strand; N L Peters; Steven M Solano; Zane R Hollingsworth; A S Menon; AS Frey; B S Spektor; E B Penney; Gabriele Schilling; Christopher A Ross; David R Borchelt; Stephen J Tapscott; Anne B Young; Jang-Ho Cha; James M Olson
Decreased expression of striatal signaling genes in a mouse model of Huntington's disease.
Human molecular genetics 2000;9(9):1259-71.
2000: S H Zaman; Angèle T Parent; A Laskey; Michael K Lee; David R Borchelt; Sangram S Sisodia; Roberto Malinow
Enhanced synaptic potentiation in transgenic mice expressing presenilin 1 familial Alzheimer's disease mutation is normalized with a benzodiazepine.
Neurobiology of disease 2000;7(1):54-63.
2000: Donald L Price; Philip C Wong; Alicja L Markowska; Michael K Lee; G Thinakaren; Don W Cleveland; Sangram S Sisodia; David R Borchelt
The value of transgenic models for the study of neurodegenerative diseases.
Annals of the New York Academy of Sciences 2000;920():179-91.
1999: Gabriele Schilling; Jonathan D Wood; K Duan; Hilda H Slunt; Victoria Gonzales; M Yamada; Jillian K Cooper; Russell L Margolis; Nancy A Jenkins; Neal G Copeland; H Takahashi; Shoji Tsuji; Donald L Price; David R Borchelt; Christopher A Ross
Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA.
Neuron 1999;24(1):275-86.
1999: TAMARA RATOVITSKI; LB Corson; J Strain; Philip C Wong; Don W Cleveland; Valeria Cizewski Culotta; David R Borchelt
Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindreds.
Human molecular genetics 1999;8(8):1451-60.
1999: Christopher A Ross; Jonathan D Wood; Gabriele Schilling; Matthew F Peters; Frederick C Nucifora; Jillian K Cooper; Alan H Sharp; Russell L Margolis; David R Borchelt
Polyglutamine pathogenesis.
Philosophical transactions of the Royal Society of London. Series B, Biological sciences 1999;354(1386):1005-11.
1999: Gabriele Schilling; Mark W Becher; Alan H Sharp; Hyder A Jinnah; K Duan; JA Kotzuk; Hilda H Slunt; TAMARA RATOVITSKI; Jillian K Cooper; Nancy A Jenkins; Neal G Copeland; Donald L Price; Christopher A Ross; David R Borchelt
Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin.
Human molecular genetics 1999;8(3):397-407.
1999: costantino iadecola; F Zhang; Kiyoshi Niwa; Christopher B Eckman; S K Turner; E Fischer; Steven G Younkin; David R Borchelt; KK Hsiao; George A Carlson
SOD1 rescues cerebral endothelial dysfunction in mice overexpressing amyloid precursor protein.
Nature neuroscience 1999;2(2):157-61.
1999: Angèle T Parent; David J Linden; Sangram S Sisodia; David R Borchelt
Synaptic transmission and hippocampal long-term potentiation in transgenic mice expressing FAD-linked presenilin 1.
Neurobiology of disease 1999;6(1):56-62.
1998: Piera Pasinelli; David R Borchelt; MK Houseweart; Don W Cleveland; Robert H Brown
Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase.
Proceedings of the National Academy of Sciences of the United States of America 1998;95(26):15763-8.
1998: David R Borchelt
Inherited neurodegenerative diseases and transgenic models.
Laboratory animal science 1998;48(6):604-10.
1998: Donald L Price; Sangram S Sisodia; David R Borchelt
Genetic neurodegenerative diseases: the human illness and transgenic models.
Science (New York, N.Y.) 1998;282(5391):1079-83.
1998: S Naruse; Gopal Thinakaran; JJ Luo; John W Kusiak; Taisuke Tomita; Takeshi Iwatsubo; X Qian; David D Ginty; Donald L Price; David R Borchelt; Philip C Wong; Sangram S Sisodia
Effects of PS1 deficiency on membrane protein trafficking in neurons.
Neuron 1998;21(5):1213-21.
1998: David R Borchelt; Philip C Wong; Sangram S Sisodia; Donald L Price
Transgenic mouse models of Alzheimer's disease and amyotrophic lateral sclerosis.
Brain pathology (Zurich, Switzerland) 1998;8(4):735-57.
1998: Donald L Price; Sangram S Sisodia; David R Borchelt
Alzheimer disease--when and why?
Nature genetics 1998;19(4):314-6.
1998: David R Borchelt; Philip C Wong; Mark W Becher; CARLOS PARDO-VILLAMIZAR; Michael K Lee; Z S Xu; Gopal Thinakaran; Nancy A Jenkins; Neal G Copeland; Sangram S Sisodia; Don W Cleveland; Donald L Price; Paul N Hoffman
Axonal transport of mutant superoxide dismutase 1 and focal axonal abnormalities in the proximal axons of transgenic mice.
Neurobiology of disease 1998;5(1):27-35.
1998: Jillian K Cooper; Gabriele Schilling; Matthew F Peters; William Joseph Herring; Alan H Sharp; Z Kaminsky; J Masone; F A Khan; M Delanoy; David R Borchelt; Valina Dawson; Ted M Dawson; Christopher A Ross
Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture.
Human molecular genetics 1998;7(5):783-90.
1998: Gopal Thinakaran; J B Regard; Christopher MLS Bouton; CL Harris; Donald L Price; David R Borchelt; Sangram S Sisodia
Stable association of presenilin derivatives and absence of presenilin interactions with APP.
Neurobiology of disease 1998;4(6):438-53.
1998: J A Davis; S Naruse; Honglin Chen; Christopher B Eckman; Steven G Younkin; Donald L Price; David R Borchelt; Sangram S Sisodia; Philip C Wong
An Alzheimer's disease-linked PS1 variant rescues the developmental abnormalities of PS1-deficient embryos.
Neuron 1998;20(3):603-9.
1998: Philip C Wong; David R Borchelt; Michael K Lee; CARLOS PARDO-VILLAMIZAR; Gopal Thinakaran; Lee J Martin; Sangram S Sisodia; Donald L Price
Familial amyotrophic lateral sclerosis and Alzheimer's disease. Transgenic models.
Advances in experimental medicine and biology 1998;446():145-59.
1998: Donald L Price; Rudolph E Tanzi; David R Borchelt; Sangram S Sisodia
Alzheimer's disease: genetic studies and transgenic models.
Annual review of genetics 1998;32():461-93.
1998: David R Borchelt
Metabolism of presenilin 1: influence of presenilin 1 on amyloid precursor protein processing.
Neurobiology of aging 1998;19(1 Suppl):S15-8.
1997: Gopal Thinakaran; CL Harris; TAMARA RATOVITSKI; F Davenport; Hilda H Slunt; Donald L Price; David R Borchelt; Sangram S Sisodia
Evidence that levels of presenilins (PS1 and PS2) are coordinately regulated by competition for limiting cellular factors.
The Journal of biological chemistry 1997;272(45):28415-22.
1997: David R Borchelt; TAMARA RATOVITSKI; J van Lare; Michael K Lee; Victoria Gonzales; Nancy A Jenkins; Neal G Copeland; Donald L Price; Sangram S Sisodia
Accelerated amyloid deposition in the brains of transgenic mice coexpressing mutant presenilin 1 and amyloid precursor proteins.
Neuron 1997;19(4):939-45.
1997: George A Carlson; David R Borchelt; A Dake; S Turner; V Danielson; J D Coffin; Christopher B Eckman; J Meiners; S P Nilsen; Steven G Younkin; KK Hsiao
Genetic modification of the phenotypes produced by amyloid precursor protein overexpression in transgenic mice.
Human molecular genetics 1997;6(11):1951-9.
1997: TAMARA RATOVITSKI; Hilda H Slunt; Gopal Thinakaran; Donald L Price; Sangram S Sisodia; David R Borchelt
Endoproteolytic processing and stabilization of wild-type and mutant presenilin.
The Journal of biological chemistry 1997;272(39):24536-41.
1997: Donald L Price; Philip C Wong; David R Borchelt; CARLOS PARDO-VILLAMIZAR; Gopal Thinakaran; AP Doan; Michael K Lee; Lee J Martin; Sangram S Sisodia
Amyotrophic lateral sclerosis and Alzheimer disease. Lessons from model systems.
Revue neurologique 1997;153(8-9):484-95.
1997: Michael K Lee; David R Borchelt; G Kim; Gopal Thinakaran; Hilda H Slunt; TAMARA RATOVITSKI; Lee J Martin; A Kittur; Sam Gandy; Allan Levey; Nancy Jenkins; Neal G Copeland; Donald L Price; Sangram S Sisodia
Hyperaccumulation of FAD-linked presenilin 1 variants in vivo.
Nature medicine 1997;3(7):756-60.
1997: Lucie I Bruijn; Mark W Becher; Michael K Lee; K L Anderson; N A Jenkins; N G Copeland; Sangram S Sisodia; Jeffrey rothstein; David R Borchelt; Donald L Price; Don W Cleveland
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.
Neuron 1997;18(2):327-38.
1996: David R Borchelt; J Davis; M Fischer; Michael K Lee; Hilda H Slunt; T Ratovitsky; J Regard; Neal G Copeland; Nancy A Jenkins; Sangram S Sisodia; Donald L Price
A vector for expressing foreign genes in the brains and hearts of transgenic mice.
Genetic analysis : biomolecular engineering 1996;13(6):159-63.
1996: AP Doan; Gopal Thinakaran; David R Borchelt; Hilda H Slunt; T Ratovitsky; M Podlisny; Dennis J Selkoe; M Seeger; Sam Gandy; Donald L Price; Sangram S Sisodia
Protein topology of presenilin 1.
Neuron 1996;17(5):1023-30.
1996: David R Borchelt; Gopal Thinakaran; Christopher B Eckman; Michael K Lee; F Davenport; T Ratovitsky; CM Prada; G Kim; S Seekins; Debra Yager; Hilda H Slunt; Rong Wang; M Seeger; Allan Levey; Sam Gandy; Neal G Copeland; Nancy A Jenkins; Donald L Price; Steven G Younkin; Sangram S Sisodia
Familial Alzheimer's disease-linked presenilin 1 variants elevate Abeta1-42/1-40 ratio in vitro and in vivo.
Neuron 1996;17(5):1005-13.
1996: Donald L Price; Mark W Becher; Philip C Wong; David R Borchelt; Michael K Lee; Sangram S Sisodia
Inherited neurodegenerative diseases and transgenic models.
Brain pathology (Zurich, Switzerland) 1996;6(4):467-80.
1996: Michael K Lee; David R Borchelt; Philip C Wong; Sangram S Sisodia; Donald L Price
Transgenic models of neurodegenerative diseases.
Current opinion in neurobiology 1996;6(5):651-60.
1996: Don W Cleveland; Lucie I Bruijn; Philip C Wong; J R Marszalek; J D Vechio; Michael K Lee; X S Xu; David R Borchelt; Sangram S Sisodia; Donald L Price
Mechanisms of selective motor neuron death in transgenic mouse models of motor neuron disease.
Neurology 1996;47(4 Suppl 2):S54-61; discussion S61-2.
1996: David R Borchelt; Sangram S Sisodia
Loss of functional prion protein: a role in prion disorders?
Chemistry & biology 1996;3(8):619-21.
1996: Gopal Thinakaran; David R Borchelt; Michael K Lee; Hilda H Slunt; L Spitzer; G Kim; T Ratovitsky; F Davenport; C Nordstedt; M Seeger; John Hardy; Allan Levey; Sam Gandy; Nancy A Jenkins; Neal G Copeland; Donald L Price; Sangram S Sisodia
Endoproteolysis of presenilin 1 and accumulation of processed derivatives in vivo.
Neuron 1996;17(1):181-90.
1996: Donald L Price; David R Borchelt; Philip C Wong; CARLOS PARDO-VILLAMIZAR; Gopal Thinakaran; Michael K Lee; Don W Cleveland; Sangram S Sisodia
Neurodegenerative diseases and model systems.
Cold Spring Harbor symposia on quantitative biology 1996;61():725-38.
1996: Donald L Price; Vassilis E Koliatsos; Philip C Wong; CARLOS PARDO-VILLAMIZAR; David R Borchelt; Michael K Lee; Don W Cleveland; John W Griffin; Paul N Hoffman; L C Cork; Sangram S Sisodia
Motor neuron disease and model systems: aetiologies, mechanisms and therapies.
Ciba Foundation symposium 1996;196():3-13; discussion 13-7.
1995: KK Hsiao; David R Borchelt; K Olson; R Johannsdottir; C Kitt; W Yunis; S Xu; Christopher B Eckman; Steven G Younkin; Donald L Price
Age-related CNS disorder and early death in transgenic FVB/N mice overexpressing Alzheimer amyloid precursor proteins.
Neuron 1995;15(5):1203-18.
1995: Philip C Wong; David R Borchelt
Motor neuron disease caused by mutations in superoxide dismutase 1.
Current opinion in neurology 1995;8(4):294-301.
1995: Philip C Wong; CARLOS PARDO-VILLAMIZAR; David R Borchelt; Michael K Lee; Neal G Copeland; Nancy A Jenkins; Sangram S Sisodia; Don W Cleveland; Donald L Price
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria.
Neuron 1995;14(6):1105-16.
1995: Shahrooz Rabizadeh; Edith Butler Gralla; David R Borchelt; R Gwinn; Joan Selverstone Valentine; Sangram S Sisodia; Philip C Wong; Michael K Lee; H Hahn; Dale E Bredesen
Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotic gene: studies in yeast and neural cells.
Proceedings of the National Academy of Sciences of the United States of America 1995;92(7):3024-8.
1995: David R Borchelt; M Guarnieri; Philip C Wong; Michael K Lee; H S Slunt; Z S Xu; Sangram S Sisodia; Donald L Price; Don W Cleveland
Superoxide dismutase 1 subunits with mutations linked to familial amyotrophic lateral sclerosis do not affect wild-type subunit function.
The Journal of biological chemistry 1995;270(7):3234-8.
1995: CARLOS PARDO-VILLAMIZAR; Z Xu; David R Borchelt; Donald L Price; Sangram S Sisodia; Don W Cleveland
Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons.
Proceedings of the National Academy of Sciences of the United States of America 1995;92(4):954-8.
1995: Sangram S Sisodia; Lee J Martin; Lary Walker; David R Borchelt; Donald L Price
Cellular and molecular biology of Alzheimer's disease and animal models.
Neuroimaging clinics of North America 1995;5(1):59-68.
1994: David R Borchelt; Michael K Lee; H S Slunt; M Guarnieri; Z S Xu; Philip C Wong; R H Brown; Donald L Price; Sangram S Sisodia; Don W Cleveland
Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity.
Proceedings of the National Academy of Sciences of the United States of America 1994;91(17):8292-6.
1994: David R Borchelt; Vassilis E Koliatsos; M Guarnieri; CARLOS PARDO-VILLAMIZAR; Sangram S Sisodia; Donald L Price
Rapid anterograde axonal transport of the cellular prion glycoprotein in the peripheral and central nervous systems.
The Journal of biological chemistry 1994;269(20):14711-4.
1993: David R Borchelt; M Rogers; Neil stahl; Glenn C Telling; Stanley B Prusiner
Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchor.
Glycobiology 1993;3(4):319-29.
1993: Donald L Price; David R Borchelt; Sangram S Sisodia
Alzheimer disease and the prion disorders amyloid beta-protein and prion protein amyloidoses.
Proceedings of the National Academy of Sciences of the United States of America 1993;90(14):6381-4.
1992: A J Raeber; David R Borchelt; Michael Scott; Stanley B Prusiner
Attempts to convert the cellular prion protein into the scrapie isoform in cell-free systems.
Journal of virology 1992;66(10):6155-63.
1992: Donald L Price; David R Borchelt; Lary Walker; Sangram S Sisodia
Toxicity of synthetic A beta peptides and modeling of Alzheimer's disease.
Neurobiology of aging 1992;13(5):623-5.
1992: David R Borchelt; A Taraboulos; Stanley B Prusiner
Evidence for synthesis of scrapie prion proteins in the endocytic pathway.
The Journal of biological chemistry 1992;267(23):16188-99.
1992: A Taraboulos; A J Raeber; David R Borchelt; D Serban; Stanley B Prusiner
Synthesis and trafficking of prion proteins in cultured cells.
Molecular biology of the cell 1992;3(8):851-63.
1990: A Taraboulos; M Rogers; David R Borchelt; M P McKinley; Michael Scott; D Serban; Stanley B Prusiner
Acquisition of protease resistance by prion proteins in scrapie-infected cells does not require asparagine-linked glycosylation.
Proceedings of the National Academy of Sciences of the United States of America 1990;87(21):8262-6.
1990: Neil stahl; David R Borchelt; Stanley B Prusiner
Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C.
Biochemistry 1990;29(22):5405-12.
1990: David R Borchelt; Michael Scott; A Taraboulos; Neil stahl; Stanley B Prusiner
Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells.
The Journal of cell biology 1990;110(3):743-52.
1989: T Haraguchi; S Fisher; S Olofsson; T Endo; Darlene Groth; AL Tarentino; David R Borchelt; David B Teplow; L Hood; Al L Burlingame
Asparagine-linked glycosylation of the scrapie and cellular prion proteins.
Archives of biochemistry and biophysics 1989;274(1):1-13.
1988: D A Butler; Michael R Scott; J M Bockman; David R Borchelt; A Taraboulos; KK Hsiao; D T Kingsbury; Stanley B Prusiner
Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins.
Journal of virology 1988;62(5):1558-64.
1988: David R Borchelt; M L Perdue
Identification of an initial site of interaction and possible helix destabilizing activity preceding initiation of protein synthesis from retrovirus RNA.
Virus research 1988;10(2-3):241-8.
1987: Neil stahl; David R Borchelt; Karen K. Hsiao Ashe; Stanley B Prusiner
Scrapie prion protein contains a phosphatidylinositol glycolipid.
Cell 1987;51(2):229-40.