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David R. Borchelt, PhD, University of Kentucky - Publications

Neuroscience University of Florida, Gainesville, Gainesville, FL, United States 
Mouse models of neurodegenerative disease

207 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any inaccuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2020 Crown A, McAlary L, Fagerli E, Brown H, Yerbury JJ, Galaleldeen A, Cashman NR, Borchelt DR, Ayers JI. Tryptophan residue 32 in human Cu-Zn superoxide dismutase modulates prion-like propagation and strain selection. Plos One. 15: e0227655. PMID 31999698 DOI: 10.1371/journal.pone.0227655  0.6
2020 Koller EJ, Gonzalez De La Cruz E, Weinrich M, Williams T, Cruz PE, Ryu D, Golde TE, Sullivan PM, Lewis J, Borchelt DR, Chakrabarty P. Intracerebral Expression of AAV-APOE4 Is Not Sufficient to Alter Tau Burden in Two Distinct Models of Tauopathy. Molecular Neurobiology. PMID 31903524 DOI: 10.1007/s12035-019-01859-4  0.44
2019 Osking Z, Ayers JI, Hildebrandt R, Skruber K, Brown H, Ryu D, Eukovich AR, Golde TE, Borchelt DR, Read TA, Vitriol EA. ALS-Linked SOD1 Mutants Enhance Neurite Outgrowth and Branching in Adult Motor Neurons. Iscience. 19: 448-449. PMID 31425915 DOI: 10.1016/j.isci.2019.08.004  0.6
2019 Gallego-Iradi MC, Strunk H, Crown AM, Davila R, Brown H, Rodriguez-Lebron E, Borchelt DR. N-terminal sequences in matrin 3 mediate phase separation into droplet-like structures that recruit TDP43 variants lacking RNA binding elements. Laboratory Investigation; a Journal of Technical Methods and Pathology. PMID 31019288 DOI: 10.1038/s41374-019-0260-7  0.44
2019 Crown AM, Roberts BL, Crosby K, Brown H, Ayers JI, Hart PJ, Borchelt DR. Experimental Mutations in Superoxide Dismutase 1 Provide Insight into Potential Mechanisms Involved in Aberrant Aggregation in Familial Amyotrophic Lateral Sclerosis. G3 (Bethesda, Md.). PMID 30622123 DOI: 10.1534/g3.118.200787  0.6
2018 Osking Z, Ayers JI, Hildebrandt R, Skruber K, Brown H, Ryu D, Eukovich AR, Golde TE, Borchelt DR, Read TA, Vitriol EA. ALS-Linked SOD1 Mutants Enhance Neurite Outgrowth and Branching in Adult Motor Neurons. Iscience. 11: 294-304. PMID 30639851 DOI: 10.1016/j.isci.2018.12.026  0.6
2018 Moloney C, Rayaprolu S, Howard J, Fromholt S, Brown H, Collins M, Cabrera M, Duffy C, Siemienski Z, Miller D, Borchelt DR, Lewis J. Analysis of spinal and muscle pathology in transgenic mice overexpressing wild-type and ALS-linked mutant MATR3. Acta Neuropathologica Communications. 6: 137. PMID 30563574 DOI: 10.1186/s40478-018-0631-0  0.6
2018 De Rossi P, Andrew RJ, Musial TF, Buggia-Prevot V, Xu G, Ponnusamy M, Ly H, Krause SV, Rice RC, de l'Estoile V, Valin T, Salem S, Despa F, Borchelt DR, Bindokas VP, et al. Aberrant accrual of BIN1 near Alzheimer's disease amyloid deposits in transgenic models. Brain Pathology (Zurich, Switzerland). PMID 30506549 DOI: 10.1111/bpa.12687  1
2018 Crosby K, Crown AM, Roberts BL, Brown H, Ayers JI, Borchelt DR. Loss of charge mutations in solvent exposed Lys residues of superoxide dismutase 1 do not induce inclusion formation in cultured cell models. Plos One. 13: e0206751. PMID 30399166 DOI: 10.1371/journal.pone.0206751  0.6
2018 Pace MC, Xu G, Fromholt S, Howard J, Crosby K, Giasson BI, Lewis J, Borchelt DR. Changes in proteome solubility indicate widespread proteostatic disruption in mouse models of neurodegenerative disease. Acta Neuropathologica. PMID 30140941 DOI: 10.1007/s00401-018-1895-y  1
2018 Pace MC, Xu G, Fromholt S, Howard J, Giasson BI, Lewis J, Borchelt DR. Differential induction of mutant SOD1 misfolding and aggregation by tau and α-synuclein pathology. Molecular Neurodegeneration. 13: 23. PMID 29776378 DOI: 10.1186/s13024-018-0253-9  1
2018 Iradi MCG, Triplett JC, Thomas JD, Davila R, Crown AM, Brown H, Lewis J, Swanson MS, Xu G, Rodriguez-Lebron E, Borchelt DR. Characterization of gene regulation and protein interaction networks for Matrin 3 encoding mutations linked to amyotrophic lateral sclerosis and myopathy. Scientific Reports. 8: 4049. PMID 29511296 DOI: 10.1038/s41598-018-21371-4  1
2017 Moloney C, Rayaprolu S, Howard J, Fromholt S, Brown H, Collins M, Cabrera M, Duffy C, Siemienski Z, Miller D, Swanson MS, Notterpek L, Borchelt DR, Lewis J. Retraction Note: Transgenic mice overexpressing the ALS-linked protein Matrin 3 develop a profound muscle phenotype. Acta Neuropathologica Communications. 5: 97. PMID 29237481 DOI: 10.1186/s40478-017-0502-0  0.6
2017 Moore BD, Martin J, de Mena L, Sanchez J, Cruz PE, Ceballos-Diaz C, Ladd TB, Ran Y, Levites Y, Kukar TL, Kurian JJ, McKenna R, Koo EH, Borchelt DR, Janus C, et al. Short Aβ peptides attenuate Aβ42 toxicity in vivo. The Journal of Experimental Medicine. PMID 29208777 DOI: 10.1084/jem.20170600  0.56
2017 Ayers JI, Giasson BI, Borchelt DR. Prion-like Spreading in Tauopathies. Biological Psychiatry. PMID 28506438 DOI: 10.1016/j.biopsych.2017.04.003  0.6
2016 Moloney C, Rayaprolu S, Howard J, Fromholt S, Brown H, Collins M, Cabrera M, Duffy C, Siemienski Z, Miller D, Swanson MS, Notterpek L, Borchelt DR, Lewis J. Transgenic mice overexpressing the ALS-linked protein Matrin 3 develop a profound muscle phenotype. Acta Neuropathologica Communications. 4: 122. PMID 27863507 DOI: 10.1186/s40478-016-0393-5  0.6
2016 Ayers JI, McMahon B, Gill S, Lelie HL, Fromholt S, Brown H, Valentine JS, Whitelegge JP, Borchelt DR. Relationship between mutant SOD1 maturation and inclusion formation in cell models. Journal of Neurochemistry. PMID 27727458 DOI: 10.1111/jnc.13864  0.6
2016 Ayers JI, Diamond J, Sari A, Fromholt S, Galaleldeen A, Ostrow LW, Glass JD, Hart PJ, Borchelt DR. Distinct conformers of transmissible misfolded SOD1 distinguish human SOD1-FALS from other forms of familial and sporadic ALS. Acta Neuropathologica. PMID 27704280 DOI: 10.1007/s00401-016-1623-4  0.6
2016 Fromholt S, Reitano C, Brown H, Lewis J, Borchelt DR. Generation of a new transgenic mouse model for assessment of tau gene silencing therapies. Alzheimer's Research & Therapy. 8: 36. PMID 27593210 DOI: 10.1186/s13195-016-0202-1  0.6
2016 Melnikova T, Park D, Becker L, Lee D, Cho E, Sayyida N, Tian J, Bandeen-Roche K, Borchelt DR, Savonenko AV. Sex-related dimorphism in dentate gyrus atrophy and behavioral phenotypes in an inducible tTa:APPsi transgenic model of Alzheimer's disease. Neurobiology of Disease. PMID 27569580 DOI: 10.1016/j.nbd.2016.08.009  0.96
2016 Liu Y, Pattamatta A, Zu T, Reid T, Bardhi O, Borchelt DR, Yachnis AT, Ranum LP. C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD. Neuron. PMID 27112499 DOI: 10.1016/j.neuron.2016.04.005  0.4
2016 Xu G, Pattamatta A, Hildago R, Pace MC, Brown H, Borchelt DR. Vulnerability of newly synthesized proteins to proteostasis stress. Journal of Cell Science. 129: 1892-901. PMID 27026526 DOI: 10.1242/jcs.176479  1
2016 Rayaprolu S, D'Alton S, Crosby K, Moloney C, Howard J, Duffy C, Cabrera M, Siemienski Z, Hernandez AR, Gallego-Iradi C, Borchelt DR, Lewis J. Heterogeneity of Matrin 3 in the developing and aging murine central nervous system. The Journal of Comparative Neurology. PMID 26878116 DOI: 10.1002/cne.23986  0.96
2015 Ayers JI, Fromholt SE, O'Neal VM, Diamond JH, Borchelt DR. Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathways. Acta Neuropathologica. PMID 26650262 DOI: 10.1007/s00401-015-1514-0  0.96
2015 Bañez-Coronel M, Ayhan F, Tarabochia AD, Zu T, Perez BA, Tusi SK, Pletnikova O, Borchelt DR, Ross CA, Margolis RL, Yachnis AT, Troncoso JC, Ranum LP. RAN Translation in Huntington Disease. Neuron. 88: 667-77. PMID 26590344 DOI: 10.1016/j.neuron.2015.10.038  0.96
2015 Xu G, Ran Y, Fromholt SE, Fu C, Yachnis AT, Golde TE, Borchelt DR. Murine Aβ over-production produces diffuse and compact Alzheimer-type amyloid deposits. Acta Neuropathologica Communications. 3: 72. PMID 26566997 DOI: 10.1186/s40478-015-0252-9  1
2015 Sacino AN, Ayers JI, Brooks MM, Chakrabarty P, Hudson VJ, Howard JK, Golde TE, Giasson BI, Borchelt DR. Non-prion-type transmission in A53T α-synuclein transgenic mice: a normal component of spinal homogenates from naïve non-transgenic mice induces robust α-synuclein pathology. Acta Neuropathologica. PMID 26541429 DOI: 10.1007/s00401-015-1505-1  0.96
2015 Gallego-Iradi MC, Clare AM, Brown HH, Janus C, Lewis J, Borchelt DR. Subcellular Localization of Matrin 3 Containing Mutations Associated with ALS and Distal Myopathy. Plos One. 10: e0142144. PMID 26528920 DOI: 10.1371/journal.pone.0142144  0.96
2015 Janus C, Flores AY, Xu G, Borchelt DR. Behavioral abnormalities in APPSwe/PS1dE9 mouse model of AD-like pathology: comparative analysis across multiple behavioral domains. Neurobiology of Aging. 36: 2519-32. PMID 26089165 DOI: 10.1016/j.neurobiolaging.2015.05.010  1
2015 Gelfand P, Smith RJ, Stavitski E, Borchelt DR, Miller LM. Characterization of Protein Structural Changes in Living Cells Using Time-Lapsed FTIR Imaging. Analytical Chemistry. 87: 6025-31. PMID 25965274 DOI: 10.1021/acs.analchem.5b00371  0.96
2015 Xu G, Fromholt S, Ayers JI, Brown H, Siemienski Z, Crosby KW, Mayer CA, Janus C, Borchelt DR. Substantially elevating the levels of αB-crystallin in spinal motor neurons of mutant SOD1 mice does not significantly delay paralysis or attenuate mutant protein aggregation. Journal of Neurochemistry. 133: 452-64. PMID 25557022 DOI: 10.1111/jnc.13022  1
2015 Xu G, Ayers JI, Roberts BL, Brown H, Fromholt S, Green C, Borchelt DR. Direct and indirect mechanisms for wild-type SOD1 to enhance the toxicity of mutant SOD1 in bigenic transgenic mice. Human Molecular Genetics. 24: 1019-35. PMID 25305079 DOI: 10.1093/hmg/ddu517  1
2015 Ayers JI, Fromholt S, Sinyavskaya O, Siemienski Z, Rosario AM, Li A, Crosby KW, Cruz PE, DiNunno NM, Janus C, Ceballos-Diaz C, Borchelt DR, Golde TE, Chakrabarty P, Levites Y. Widespread and efficient transduction of spinal cord and brain following neonatal AAV injection and potential disease modifying effect in ALS mice. Molecular Therapy : the Journal of the American Society of Gene Therapy. 23: 53-62. PMID 25228069 DOI: 10.1038/mt.2014.180  0.96
2014 Borchelt DR, Ross CA. Gabriele schilling (september 5, 1968-july 4, 2014. Journal of Huntington's Disease. 3: 225-7. PMID 25300326 DOI: 10.3233/JHD-149005  0.96
2014 Ayers JI, Fromholt S, Koch M, DeBosier A, McMahon B, Xu G, Borchelt DR. Experimental transmissibility of mutant SOD1 motor neuron disease. Acta Neuropathologica. 128: 791-803. PMID 25262000 DOI: 10.1007/s00401-014-1342-7  1
2014 Brown HH, Borchelt DR. Analysis of mutant SOD1 electrophoretic mobility by Blue Native gel electrophoresis; evidence for soluble multimeric assemblies. Plos One. 9: e104583. PMID 25121776 DOI: 10.1371/journal.pone.0104583  0.96
2014 Tebbenkamp AT, Xu G, Siemienski ZB, Janus C, Fromholt SE, Brown HH, Swing D, Tessarollo L, Borchelt DR. Experimental mutagenesis of huntingtin to map cleavage sites: different outcomes in cell and mouse models. Journal of Huntington's Disease. 3: 73-86. PMID 25062766 DOI: 10.3233/JHD-130087  0.96
2014 Sacino AN, Brooks M, Thomas MA, McKinney AB, Lee S, Regenhardt RW, McGarvey NH, Ayers JI, Notterpek L, Borchelt DR, Golde TE, Giasson BI. Intramuscular injection of α-synuclein induces CNS α-synuclein pathology and a rapid-onset motor phenotype in transgenic mice. Proceedings of the National Academy of Sciences of the United States of America. 111: 10732-7. PMID 25002524 DOI: 10.1073/pnas.1321785111  0.96
2014 Bourassa MW, Brown HH, Borchelt DR, Vogt S, Miller LM. Metal-deficient aggregates and diminished copper found in cells expressing SOD1 mutations that cause ALS. Frontiers in Aging Neuroscience. 6: 110. PMID 24982630 DOI: 10.3389/fnagi.2014.00110  0.96
2014 Ayers JI, Xu G, Pletnikova O, Troncoso JC, Hart PJ, Borchelt DR. Conformational specificity of the C4F6 SOD1 antibody; low frequency of reactivity in sporadic ALS cases. Acta Neuropathologica Communications. 2: 55. PMID 24887207 DOI: 10.1186/2051-5960-2-55  0.96
2014 March A, Borchelt D, Golde T, Janus C. Differences in memory development among C57BL/6NCrl, 129S2/SvPasCrl, and FVB/NCrl mice after delay and trace fear conditioning. Comparative Medicine. 64: 4-12. PMID 24672832  0.96
2014 Felsenstein KM, Candelario KM, Steindler DA, Borchelt DR. Regenerative medicine in Alzheimer's disease. Translational Research : the Journal of Laboratory and Clinical Medicine. 163: 432-8. PMID 24286919 DOI: 10.1016/j.trsl.2013.11.001  0.96
2014 Ayers J, Lelie H, Workman A, Prudencio M, Brown H, Fromholt S, Valentine J, Whitelegge J, Borchelt D. Distinctive features of the D101N and D101G variants of superoxide dismutase 1; two mutations that produce rapidly progressing motor neuron disease. Journal of Neurochemistry. 128: 305-14. PMID 24032979 DOI: 10.1111/jnc.12451  0.96
2013 Qualls DA, Crosby K, Brown H, Borchelt DR. An analysis of interactions between fluorescently-tagged mutant and wild-type SOD1 in intracellular inclusions. Plos One. 8: e83981. PMID 24391857 DOI: 10.1371/journal.pone.0083981  0.96
2013 Qualls DA, Prudencio M, Roberts BL, Crosby K, Brown H, Borchelt DR. Features of wild-type human SOD1 limit interactions with misfolded aggregates of mouse G86R Sod1. Molecular Neurodegeneration. 8: 46. PMID 24341866 DOI: 10.1186/1750-1326-8-46  0.96
2013 Chakrabarty P, Rosario A, Cruz P, Siemienski Z, Ceballos-Diaz C, Crosby K, Jansen K, Borchelt DR, Kim JY, Jankowsky JL, Golde TE, Levites Y. Capsid serotype and timing of injection determines AAV transduction in the neonatal mice brain. Plos One. 8: e67680. PMID 23825679 DOI: 10.1371/journal.pone.0067680  0.96
2013 Chan PK, Chattopadhyay M, Sharma S, Souda P, Gralla EB, Borchelt DR, Whitelegge JP, Valentine JS. Structural similarity of wild-type and ALS-mutant superoxide dismutase-1 fibrils using limited proteolysis and atomic force microscopy. Proceedings of the National Academy of Sciences of the United States of America. 110: 10934-9. PMID 23781106 DOI: 10.1073/pnas.1309613110  0.96
2013 Rutherford NJ, Lewis J, Clippinger AK, Thomas MA, Adamson J, Cruz PE, Cannon A, Xu G, Golde TE, Shaw G, Borchelt DR, Giasson BI. Unbiased screen reveals ubiquilin-1 and -2 highly associated with huntingtin inclusions. Brain Research. 1524: 62-73. PMID 23774650 DOI: 10.1016/j.brainres.2013.06.006  0.96
2013 Price AR, Xu G, Siemienski ZB, Smithson LA, Borchelt DR, Golde TE, Felsenstein KM. Comment on "ApoE-directed therapeutics rapidly clear β-amyloid and reverse deficits in AD mouse models". Science (New York, N.Y.). 340: 924-d. PMID 23704553 DOI: 10.1126/science.1234089  1
2013 Clippinger AK, D'Alton S, Lin WL, Gendron TF, Howard J, Borchelt DR, Cannon A, Carlomagno Y, Chakrabarty P, Cook C, Golde TE, Levites Y, Ranum L, Schultheis PJ, Xu G, et al. Robust cytoplasmic accumulation of phosphorylated TDP-43 in transgenic models of tauopathy. Acta Neuropathologica. 126: 39-50. PMID 23666556 DOI: 10.1007/s00401-013-1123-8  0.96
2013 Kim J, Chakrabarty P, Hanna A, March A, Dickson DW, Borchelt DR, Golde T, Janus C. Normal cognition in transgenic BRI2-Aβ mice. Molecular Neurodegeneration. 8: 15. PMID 23663320 DOI: 10.1186/1750-1326-8-15  0.96
2013 Golde TE, Borchelt DR, Giasson BI, Lewis J. Thinking laterally about neurodegenerative proteinopathies. The Journal of Clinical Investigation. 123: 1847-55. PMID 23635781 DOI: 10.1172/JCI66029  0.96
2013 Xu G, Stevens SM, Moore BD, McClung S, Borchelt DR. Cytosolic proteins lose solubility as amyloid deposits in a transgenic mouse model of Alzheimer-type amyloidosis. Human Molecular Genetics. 22: 2765-74. PMID 23512986 DOI: 10.1093/hmg/ddt121  1
2013 Melnikova T, Fromholt S, Kim H, Lee D, Xu G, Price A, Moore BD, Golde TE, Felsenstein KM, Savonenko A, Borchelt DR. Reversible pathologic and cognitive phenotypes in an inducible model of Alzheimer-amyloidosis. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 33: 3765-79. PMID 23447589 DOI: 10.1523/JNEUROSCI.4251-12.2013  1
2012 Tebbenkamp AT, Crosby KW, Siemienski ZB, Brown HH, Golde TE, Borchelt DR. Analysis of proteolytic processes and enzymatic activities in the generation of huntingtin n-terminal fragments in an HEK293 cell model. Plos One. 7: e50750. PMID 23236391 DOI: 10.1371/journal.pone.0050750  0.96
2012 Xu G, Stevens SM, Kobeissy F, Kobiessy F, Brown H, McClung S, Gold MS, Borchelt DR. Identification of proteins sensitive to thermal stress in human neuroblastoma and glioma cell lines. Plos One. 7: e49021. PMID 23145051 DOI: 10.1371/journal.pone.0049021  1
2012 Roberts BL, Patel K, Brown HH, Borchelt DR. Role of disulfide cross-linking of mutant SOD1 in the formation of inclusion-body-like structures. Plos One. 7: e47838. PMID 23118898 DOI: 10.1371/journal.pone.0047838  0.96
2012 Shi Y, Mowery RA, Ashley J, Hentz M, Ramirez AJ, Bilgicer B, Slunt-Brown H, Borchelt DR, Shaw BF. Abnormal SDS-PAGE migration of cytosolic proteins can identify domains and mechanisms that control surfactant binding. Protein Science : a Publication of the Protein Society. 21: 1197-209. PMID 22692797 DOI: 10.1002/pro.2107  0.96
2012 Xu G, Green CC, Fromholt SE, Borchelt DR. Reduction of low-density lipoprotein receptor-related protein (LRP1) in hippocampal neurons does not proportionately reduce, or otherwise alter, amyloid deposition in APPswe/PS1dE9 transgenic mice. Alzheimer's Research & Therapy. 4: 12. PMID 22537779 DOI: 10.1186/alzrt110  0.96
2012 Njie eG, Kantorovich S, Astary GW, Green C, Zheng T, Semple-Rowland SL, Steindler DA, Sarntinoranont M, Streit WJ, Borchelt DR. A preclinical assessment of neural stem cells as delivery vehicles for anti-amyloid therapeutics. Plos One. 7: e34097. PMID 22496779 DOI: 10.1371/journal.pone.0034097  0.96
2012 Prudencio M, Lelie H, Brown HH, Whitelegge JP, Valentine JS, Borchelt DR. 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. 121: 475-85. PMID 22332887 DOI: 10.1111/j.1471-4159.2012.07690.x  0.96
2012 Njie EG, Boelen E, Stassen FR, Steinbusch HW, Borchelt DR, Streit WJ. Ex vivo cultures of microglia from young and aged rodent brain reveal age-related changes in microglial function. Neurobiology of Aging. 33: 195.e1-12. PMID 20580465 DOI: 10.1016/j.neurobiolaging.2010.05.008  0.96
2011 Prudencio M, Borchelt DR. Superoxide dismutase 1 encoding mutations linked to ALS adopts a spectrum of misfolded states. Molecular Neurodegeneration. 6: 77. PMID 22094223 DOI: 10.1186/1750-1326-6-77  0.96
2011 Tebbenkamp AT, Green C, Xu G, Denovan-Wright EM, Rising AC, Fromholt SE, Brown HH, Swing D, Mandel RJ, Tessarollo L, Borchelt DR. Transgenic mice expressing caspase-6-derived N-terminal fragments of mutant huntingtin develop neurologic abnormalities with predominant cytoplasmic inclusion pathology composed largely of a smaller proteolytic derivative. Human Molecular Genetics. 20: 2770-82. PMID 21515588 DOI: 10.1093/hmg/ddr176  0.96
2011 Chen KA, Cruz PE, Lanuto DJ, Flotte TR, Borchelt DR, Srivastava A, Zhang J, Steindler DA, Zheng T. Cellular fusion for gene delivery to SCA1 affected Purkinje neurons. Molecular and Cellular Neurosciences. 47: 61-70. PMID 21420496 DOI: 10.1016/j.mcn.2011.03.003  0.96
2011 Tebbenkamp AT, Swing D, Tessarollo L, Borchelt DR. Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment. Human Molecular Genetics. 20: 1633-42. PMID 21307087 DOI: 10.1093/hmg/ddr040  0.96
2011 Lelie HL, Liba A, Bourassa MW, Chattopadhyay M, Chan PK, Gralla EB, Miller LM, Borchelt DR, Valentine JS, Whitelegge JP. Copper and zinc metallation status of copper-zinc superoxide dismutase from amyotrophic lateral sclerosis transgenic mice. The Journal of Biological Chemistry. 286: 2795-806. PMID 21068388 DOI: 10.1074/jbc.M110.186999  0.96
2011 Liu Y, Lee MK, James MM, Price DL, Borchelt DR, Troncoso JC, Oh ES. Passive (amyloid-β) immunotherapy attenuates monoaminergic axonal degeneration in the AβPPswe/PS1dE9 mice. Journal of Alzheimer's Disease : Jad. 23: 271-9. PMID 20966549 DOI: 10.3233/JAD-2010-101602  0.96
2010 Tebbenkamp AT, Borchelt DR. Analysis of chaperone mRNA expression in the adult mouse brain by meta analysis of the Allen Brain Atlas. Plos One. 5: e13675. PMID 21060842 DOI: 10.1371/journal.pone.0013675  0.96
2010 Prudencio M, Durazo A, Whitelegge JP, Borchelt DR. An examination of wild-type SOD1 in modulating the toxicity and aggregation of ALS-associated mutant SOD1. Human Molecular Genetics. 19: 4774-89. PMID 20871097 DOI: 10.1093/hmg/ddq408  0.96
2010 Karch CM, Borchelt DR. Aggregation modulating elements in mutant human superoxide dismutase 1. Archives of Biochemistry and Biophysics. 503: 175-82. PMID 20682279 DOI: 10.1016/  0.96
2010 Klevytska AM, Tebbenkamp AT, Savonenko AV, Borchelt DR. Partial depletion of CREB-binding protein reduces life expectancy in a mouse model of Huntington disease. Journal of Neuropathology and Experimental Neurology. 69: 396-404. PMID 20448484 DOI: 10.1097/NEN.0b013e3181d6c436  0.96
2010 Smith WW, Liu Z, Liang Y, Masuda N, Swing DA, Jenkins NA, Copeland NG, Troncoso JC, Pletnikov M, Dawson TM, Martin LJ, Moran TH, Lee MK, Borchelt DR, Ross CA. Synphilin-1 attenuates neuronal degeneration in the A53T alpha-synuclein transgenic mouse model. Human Molecular Genetics. 19: 2087-98. PMID 20185556 DOI: 10.1093/hmg/ddq086  0.96
2010 Karch CM, Borchelt DR. 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. 113: 1092-100. PMID 20067574 DOI: 10.1111/j.1471-4159.2010.06572.x  0.96
2010 Winkler DD, Prudencio M, Karch C, Borchelt DR, Hart PJ. Copper-Zinc Superoxide Dismutase, Its Copper Chaperone, and Familial Amyotrophic Lateral Sclerosis Protein Misfolding Diseases: Current and Emerging Principles and Therapies. 381-401. DOI: 10.1002/9780470572702.ch17  0.96
2009 Tebbenkamp AT, Borchelt DR. Protein aggregate characterization in models of neurodegenerative disease. Methods in Molecular Biology (Clifton, N.J.). 566: 85-91. PMID 20058166 DOI: 10.1007/978-1-59745-562-6_6  0.96
2009 Boylan K, Yang C, Crook J, Overstreet K, Heckman M, Wang Y, Borchelt D, Shaw G. Immunoreactivity of the phosphorylated axonal neurofilament H subunit (pNF-H) in blood of ALS model rodents and ALS patients: evaluation of blood pNF-H as a potential ALS biomarker. Journal of Neurochemistry. 111: 1182-91. PMID 19765193 DOI: 10.1111/j.1471-4159.2009.06386.x  0.96
2009 Seetharaman SV, Prudencio M, Karch C, Holloway SP, Borchelt DR, Hart PJ. Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis. Experimental Biology and Medicine (Maywood, N.J.). 234: 1140-54. PMID 19596823 DOI: 10.3181/0903-MR-104  0.96
2009 Prudencio M, Hart PJ, Borchelt DR, Andersen PM. Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease. Human Molecular Genetics. 18: 3217-26. PMID 19483195 DOI: 10.1093/hmg/ddp260  0.96
2009 Karch CM, Prudencio M, Winkler DD, Hart PJ, Borchelt DR. 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. 106: 7774-9. PMID 19416874 DOI: 10.1073/pnas.0902505106  0.96
2009 Winkler DD, Schuermann JP, Cao X, Holloway SP, Borchelt DR, Carroll MC, Proescher JB, Culotta VC, Hart PJ. Structural and biophysical properties of the pathogenic SOD1 variant H46R/H48Q. Biochemistry. 48: 3436-47. PMID 19227972 DOI: 10.1021/bi8021735  0.96
2009 Prudencio M, Durazo A, Whitelegge JP, Borchelt DR. Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme. Journal of Neurochemistry. 108: 1009-18. PMID 19077113 DOI: 10.1111/j.1471-4159.2008.05839.x  0.96
2009 Oh ES, Savonenko AV, King JF, Fangmark Tucker SM, Rudow GL, Xu G, Borchelt DR, Troncoso JC. Amyloid precursor protein increases cortical neuron size in transgenic mice. Neurobiology of Aging. 30: 1238-44. PMID 18304698 DOI: 10.1016/j.neurobiolaging.2007.12.024  1
2008 Liu Y, Yoo MJ, Savonenko A, Stirling W, Price DL, Borchelt DR, Mamounas L, Lyons WE, Blue ME, Lee MK. 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. 28: 13805-14. PMID 19091971 DOI: 10.1523/JNEUROSCI.4218-08.2008  0.96
2008 Xu G, Karch C, Li N, Lin N, Fromholt D, Gonzales V, Borchelt DR. Receptor-associated protein (RAP) plays a central role in modulating Abeta deposition in APP/PS1 transgenic mice. Plos One. 3: e3159. PMID 18776935 DOI: 10.1371/journal.pone.0003159  1
2008 Muller FL, Liu Y, Jernigan A, Borchelt D, Richardson A, Van Remmen H. MnSOD deficiency has a differential effect on disease progression in two different ALS mutant mouse models Muscle and Nerve. 38: 1173-1183. PMID 18720509 DOI: 10.1002/mus.21049  0.96
2008 Chang Y, Kong Q, Shan X, Tian G, Ilieva H, Cleveland DW, Rothstein JD, Borchelt DR, Wong PC, Lin CL. Messenger RNA oxidation occurs early in disease pathogenesis and promotes motor neuron degeneration in ALS. Plos One. 3: e2849. PMID 18682740 DOI: 10.1371/journal.pone.0002849  0.96
2008 Karch CM, Borchelt DR. A limited role for disulfide cross-linking in the aggregation of mutant SOD1 linked to familial amyotrophic lateral sclerosis. The Journal of Biological Chemistry. 283: 13528-37. PMID 18316367 DOI: 10.1074/jbc.M800564200  0.96
2008 Shaw BF, Lelie HL, Durazo A, Nersissian AM, Xu G, Chan PK, Gralla EB, Tiwari A, Hayward LJ, Borchelt DR, Valentine JS, Whitelegge JP. Detergent-insoluble aggregates associated with amyotrophic lateral sclerosis in transgenic mice contain primarily full-length, unmodified superoxide dismutase-1. The Journal of Biological Chemistry. 283: 8340-50. PMID 18192269 DOI: 10.1074/jbc.M707751200  0.96
2008 Tucker SM, Borchelt DR, Troncoso JC. 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. 67: 30-40. PMID 18091561 DOI: 10.1097/nen.0b013e31815f38d2  0.96
2008 Wang J, Martin E, Gonzales V, Borchelt DR, Lee MK. Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases. Neurobiology of Aging. 29: 586-97. PMID 17316906 DOI: 10.1016/j.neurobiolaging.2006.11.009  0.96
2008 Borchelt DR, Savonenko AV. Chapter 5.5 Transgenic mouse models of Alzheimer's disease and episodic-like memory Handbook of Behavioral Neuroscience. 18: 553-573. DOI: 10.1016/S1569-7339(08)00230-0  0.96
2007 Ratovitski T, Nakamura M, D'Ambola J, Chighladze E, Liang Y, Wang W, Graham R, Hayden MR, Borchelt DR, Hirschhorn RR, Ross CA. N-terminal proteolysis of full-length mutant huntingtin in an inducible PC12 cell model of Huntington's disease. Cell Cycle (Georgetown, Tex.). 6: 2970-81. PMID 18156806  0.96
2007 Krewski D, Yokel RA, Nieboer E, Borchelt D, Cohen J, Harry J, Kacew S, Lindsay J, Mahfouz AM, Rondeau V. Human health risk assessment for aluminium, aluminium oxide, and aluminium hydroxide Journal of Toxicology and Environmental Health - Part B: Critical Reviews. 10: 1-269. PMID 18085482 DOI: 10.1080/10937400701597766  0.96
2007 Gary DS, Davidson A, Milhavet O, Slunt H, Borchelt DR. Investigation of RNA interference to suppress expression of full-length and fragment human huntingtin. Neuromolecular Medicine. 9: 145-55. PMID 17627034 DOI: 10.1007/s12017-007-0004-5  0.96
2007 Verret L, Jankowsky JL, Xu GM, Borchelt DR, Rampon C. 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. 27: 6771-80. PMID 17581964 DOI: 10.1523/JNEUROSCI.5564-06.2007  0.8
2007 Jankowsky JL, Younkin LH, Gonzales V, Fadale DJ, Slunt HH, Lester HA, Younkin SG, Borchelt DR. Rodent A beta modulates the solubility and distribution of amyloid deposits in transgenic mice. The Journal of Biological Chemistry. 282: 22707-20. PMID 17556372 DOI: 10.1074/jbc.M611050200  0.96
2007 Schilling G, Klevytska A, Tebbenkamp AT, Juenemann K, Cooper J, Gonzales V, Slunt H, Poirer M, Ross CA, Borchelt DR. Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models. Journal of Neuropathology and Experimental Neurology. 66: 313-20. PMID 17413322 DOI: 10.1097/nen.0b013e318040b2c8  0.96
2007 Wang J, Caruano-Yzermans A, Rodriguez A, Scheurmann JP, Slunt HH, Cao X, Gitlin J, Hart PJ, Borchelt DR. 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. 282: 345-52. PMID 17092942 DOI: 10.1074/jbc.M604503200  0.96
2006 Borchelt DR. Amyotrophic lateral sclerosis--are microglia killing motor neurons? The New England Journal of Medicine. 355: 1611-3. PMID 17035656 DOI: 10.1056/NEJMcibr064358  0.96
2006 Zamora E, Handisurya A, Shafti-Keramat S, Borchelt D, Rudow G, Conant K, Cox C, Troncoso JC, Kirnbauer R. Papillomavirus-like particles are an effective platform for amyloid-beta immunization in rabbits and transgenic mice. Journal of Immunology (Baltimore, Md. : 1950). 177: 2662-70. PMID 16888028  0.96
2006 Wang J, Xu G, Borchelt DR. Mapping superoxide dismutase 1 domains of non-native interaction: roles of intra- and intermolecular disulfide bonding in aggregation. Journal of Neurochemistry. 96: 1277-88. PMID 16441516 DOI: 10.1111/j.1471-4159.2005.03642.x  1
2006 Tanaka Y, Igarashi S, Nakamura M, Gafni J, Torcassi C, Schilling G, Crippen D, Wood JD, Sawa A, Jenkins NA, Copeland NG, Borchelt DR, Ross CA, Ellerby LM. 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. 21: 381-91. PMID 16150600 DOI: 10.1016/j.nbd.2005.07.014  0.96
2005 Laird FM, Cai H, Savonenko AV, Farah MH, He K, Melnikova T, Wen H, Chiang HC, Xu G, Koliatsos VE, Borchelt DR, Price DL, Lee HK, Wong PC. 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. 25: 11693-709. PMID 16354928 DOI: 10.1523/JNEUROSCI.2766-05.2005  1
2005 Jankowsky JL, Slunt HH, Gonzales V, Savonenko AV, Wen JC, Jenkins NA, Copeland NG, Younkin LH, Lester HA, Younkin SG, Borchelt DR. Persistent amyloidosis following suppression of Abeta production in a transgenic model of Alzheimer disease. Plos Medicine. 2: e355. PMID 16279840 DOI: 10.1371/journal.pmed.0020355  0.96
2005 Jenkins BG, Andreassen OA, Dedeoglu A, Leavitt B, Hayden M, Borchelt D, Ross CA, Ferrante RJ, Beal MF. 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. 95: 553-62. PMID 16135087 DOI: 10.1111/j.1471-4159.2005.03411.x  0.96
2005 Wang J, Xu G, Slunt HH, Gonzales V, Coonfield M, Fromholt D, Copeland NG, Jenkins NA, Borchelt DR. Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA. Neurobiology of Disease. 20: 943-52. PMID 16046140 DOI: 10.1016/j.nbd.2005.06.005  1
2005 Wang J, Xu G, Li H, Gonzales V, Fromholt D, Karch C, Copeland NG, Jenkins NA, Borchelt DR. Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: alphaB-crystallin modulates aggregation. Human Molecular Genetics. 14: 2335-47. PMID 16000321 DOI: 10.1093/hmg/ddi236  1
2005 Jankowsky JL, Melnikova T, Fadale DJ, Xu GM, Slunt HH, Gonzales V, Younkin LH, Younkin SG, Borchelt DR, Savonenko AV. 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. 25: 5217-24. PMID 15917461 DOI: 10.1523/JNEUROSCI.5080-04.2005  0.88
2005 Savonenko A, Xu GM, Melnikova T, Morton JL, Gonzales V, Wong MP, Price DL, Tang F, Markowska AL, Borchelt DR. 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. 18: 602-17. PMID 15755686 DOI: 10.1016/j.nbd.2004.10.022  0.88
2005 Price DL, Borchelt DR, Lee MK, Wong PC. Selected genetically engineered models relevant to human neurodegenerative disease Neurodegenerative Diseases: Neurobiology, Pathogenesis and Therapeutics. 176-195. DOI: 10.1017/CBO9780511544873.016  0.96
2005 Price DL, Borchelt DR, Wong PC. Alzheimer's Disease. Clinical Features, Neuropathological and Biochemical Abnormalities, Genetics, Models, and Experimental Therapeutics From Neuroscience to Neurology. 445-458. DOI: 10.1016/B978-012738903-5/50028-X  0.96
2005 Schilling G, Ross CA, Borchelt DR. Transgenic rodent models of huntington disease Movement Disorders. 309-316. DOI: 10.1016/B978-012088382-0/50027-X  0.96
2004 Jankowsky JL, Slunt HH, Gonzales V, Jenkins NA, Copeland NG, Borchelt DR. APP processing and amyloid deposition in mice haplo-insufficient for presenilin 1. Neurobiology of Aging. 25: 885-92. PMID 15212842 DOI: 10.1016/j.neurobiolaging.2003.09.008  0.96
2004 Schilling G, Savonenko AV, Klevytska A, Morton JL, Tucker SM, Poirier M, Gale A, Chan N, Gonzales V, Slunt HH, Coonfield ML, Jenkins NA, Copeland NG, Ross CA, Borchelt DR. Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice. Human Molecular Genetics. 13: 1599-610. PMID 15190011 DOI: 10.1093/hmg/ddh175  0.96
2004 Schilling G, Savonenko AV, Coonfield ML, Morton JL, Vorovich E, Gale A, Neslon C, Chan N, Eaton M, Fromholt D, Ross CA, Borchelt DR. Environmental, pharmacological, and genetic modulation of the HD phenotype in transgenic mice. Experimental Neurology. 187: 137-49. PMID 15081595 DOI: 10.1016/j.expneurol.2004.01.003  0.96
2004 Jankowsky JL, Fadale DJ, Anderson J, Xu GM, Gonzales V, Jenkins NA, Copeland NG, Lee MK, Younkin LH, Wagner SL, Younkin SG, Borchelt DR. 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. 13: 159-70. PMID 14645205 DOI: 10.1093/hmg/ddh019  0.8
2003 Jankowsky JL, Xu G, Fromholt D, Gonzales V, Borchelt DR. Environmental enrichment exacerbates amyloid plaque formation in a transgenic mouse model of Alzheimer disease. Journal of Neuropathology and Experimental Neurology. 62: 1220-7. PMID 14692698  1
2003 Sheng JG, Bora SH, Xu G, Borchelt DR, Price DL, Koliatsos VE. Lipopolysaccharide-induced-neuroinflammation increases intracellular accumulation of amyloid precursor protein and amyloid beta peptide in APPswe transgenic mice. Neurobiology of Disease. 14: 133-45. PMID 13678674 DOI: 10.1016/S0969-9961(03)00069-X  0.96
2003 Wang J, Slunt H, Gonzales V, Fromholt D, Coonfield M, Copeland NG, Jenkins NA, Borchelt DR. Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature. Human Molecular Genetics. 12: 2753-64. PMID 12966034 DOI: 10.1093/hmg/ddg312  0.96
2003 Savonenko AV, Xu GM, Price DL, Borchelt DR, Markowska AL. Normal cognitive behavior in two distinct congenic lines of transgenic mice hyperexpressing mutant APP SWE. Neurobiology of Disease. 12: 194-211. PMID 12742740  0.88
2003 Kamenetz F, Tomita T, Hsieh H, Seabrook G, Borchelt D, Iwatsubo T, Sisodia S, Malinow R. APP processing and synaptic function. Neuron. 37: 925-37. PMID 12670422 DOI: 10.1016/S0896-6273(03)00124-7  0.96
2003 Schilling G, Borchelt DR. Identifying new therapeutics for Huntington's disease Clinical Neuroscience Research. 3: 179-186. DOI: 10.1016/S1566-2772(03)00060-4  0.96
2002 Kratz JE, Stearns D, Huso DL, Slunt HH, Price DL, Borchelt DR, Eberhart CG. Expression of stabilized beta-catenin in differentiated neurons of transgenic mice does not result in tumor formation. Bmc Cancer. 2: 33. PMID 12460454 DOI: 10.1186/1471-2407-2-33  0.96
2002 Xu G, Gonzales V, Borchelt DR. Abeta deposition does not cause the aggregation of endogenous tau in transgenic mice. Alzheimer Disease and Associated Disorders. 16: 196-201. PMID 12218652  1
2002 Xu G, Gonzales V, Borchelt DR. Rapid detection of protein aggregates in the brains of Alzheimer patients and transgenic mouse models of amyloidosis. Alzheimer Disease and Associated Disorders. 16: 191-5. PMID 12218651  1
2002 Jankowsky JL, Savonenko A, Schilling G, Wang J, Xu G, Borchelt DR. Transgenic mouse models of neurodegenerative disease: opportunities for therapeutic development. Current Neurology and Neuroscience Reports. 2: 457-64. PMID 12169227  1
2002 Luthi-Carter R, Strand AD, Hanson SA, Kooperberg C, Schilling G, La Spada AR, Merry DE, Young AB, Ross CA, Borchelt DR, Olson JM. Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects. Human Molecular Genetics. 11: 1927-37. PMID 12165555  0.96
2002 Wang J, Xu G, Gonzales V, Coonfield M, Fromholt D, Copeland NG, Jenkins NA, Borchelt DR. Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site. Neurobiology of Disease. 10: 128-38. PMID 12127151  1
2002 Wong PC, Cai H, Borchelt DR, Price DL. Genetically engineered mouse models of neurodegenerative diseases. Nature Neuroscience. 5: 633-9. PMID 12085093 DOI: 10.1038/nn0702-633  0.96
2002 Wheeler VC, Gutekunst CA, Vrbanac V, Lebel LA, Schilling G, Hersch S, Friedlander RM, Gusella JF, Vonsattel JP, Borchelt DR, MacDonald ME. Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Human Molecular Genetics. 11: 633-40. PMID 11912178  0.96
2002 Wang J, Xu G, Borchelt DR. High molecular weight complexes of mutant superoxide dismutase 1: age-dependent and tissue-specific accumulation. Neurobiology of Disease. 9: 139-48. PMID 11895367 DOI: 10.1006/nbdi.2001.0471  1
2002 Borchelt DR, Lee MK, Gonzales V, Slunt HH, Ratovitski T, Jenkins NA, Copeland NG, Price DL, Sisodia SS. Accumulation of proteolytic fragments of mutant presenilin 1 and accelerated amyloid deposition are co-regulated in transgenic mice. Neurobiology of Aging. 23: 171-7. PMID 11804700 DOI: 10.1016/S0197-4580(01)00280-9  0.96
2001 Wong PC, Cai H, Borchelt DR, Price DL. Genetically engineered models relevant to neurodegenerative disorders: their value for understanding disease mechanisms and designing/testing experimental therapeutics. Journal of Molecular Neuroscience : Mn. 17: 233-57. PMID 11816796 DOI: 10.1385/JMN:17:2:233  0.96
2001 Vehmas AK, Borchelt DR, Price DL, McCarthy D, Wills-Karp M, Peper MJ, Rudow G, Luyinbazi J, Siew LT, Troncoso JC. 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. 20: 713-21. PMID 11788049 DOI: 10.1089/10445490152717578  0.96
2001 Schilling G, Coonfield ML, Ross CA, Borchelt DR. Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model. Neuroscience Letters. 315: 149-53. PMID 11716985 DOI: 10.1016/S0304-3940(01)02326-6  0.96
2001 Lesuisse C, Xu G, Anderson J, Wong M, Jankowsky J, Holtz G, Gonzalez V, Wong PC, Price DL, Tang F, Wagner S, Borchelt DR. Hyper-expression of human apolipoprotein E4 in astroglia and neurons does not enhance amyloid deposition in transgenic mice. Human Molecular Genetics. 10: 2525-37. PMID 11709540  0.96
2001 Andreassen OA, Ferrante RJ, Huang HM, Dedeoglu A, Park L, Ferrante KL, Kwon J, Borchelt DR, Ross CA, Gibson GE, Beal MF. Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease. Annals of Neurology. 50: 112-7. PMID 11456300 DOI: 10.1002/ana.1085  0.96
2001 Andreassen OA, Dedeoglu A, Ferrante RJ, Jenkins BG, Ferrante KL, Thomas M, Friedlich A, Browne SE, Schilling G, Borchelt DR, Hersch SM, Ross CA, Beal MF. Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease. Neurobiology of Disease. 8: 479-91. PMID 11447996 DOI: 10.1006/nbdi.2001.0406  0.96
2001 Schilling G, Jinnah HA, Gonzales V, Coonfield ML, Kim Y, Wood JD, Price DL, Li XJ, Jenkins N, Copeland N, Moran T, Ross CA, Borchelt DR. Distinct behavioral and neuropathological abnormalities in transgenic mouse models of HD and DRPLA. Neurobiology of Disease. 8: 405-18. PMID 11442350 DOI: 10.1006/nbdi.2001.0385  0.96
2001 Jankowsky JL, Slunt HH, Ratovitski T, Jenkins NA, Copeland NG, Borchelt DR. Co-expression of multiple transgenes in mouse CNS: a comparison of strategies. Biomolecular Engineering. 17: 157-65. PMID 11337275 DOI: 10.1016/S1389-0344(01)00067-3  0.96
2001 Cai H, Wang Y, McCarthy D, Wen H, Borchelt DR, Price DL, Wong PC. BACE1 is the major beta-secretase for generation of Abeta peptides by neurons. Nature Neuroscience. 4: 233-4. PMID 11224536 DOI: 10.1038/85064  0.96
2000 Price DL, Wong PC, Markowska AL, Lee MK, Thinakaren G, Cleveland DW, Sisodia SS, Borchelt DR. The value of transgenic models for the study of neurodegenerative diseases. Annals of the New York Academy of Sciences. 920: 179-91. PMID 11193148  0.96
2000 Takahashi M, Doré S, Ferris CD, Tomita T, Sawa A, Wolosker H, Borchelt DR, Iwatsubo T, Kim SH, Thinakaran G, Sisodia SS, Snyder SH. Amyloid precursor proteins inhibit heme oxygenase activity and augment neurotoxicity in Alzheimer's disease. Neuron. 28: 461-73. PMID 11144356  0.96
2000 Luthi-Carter R, Strand A, Peters NL, Solano SM, Hollingsworth ZR, Menon AS, Frey AS, Spektor BS, Penney EB, Schilling G, Ross CA, Borchelt DR, Tapscott SJ, Young AB, Cha JH, et al. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease. Human Molecular Genetics. 9: 1259-71. PMID 10814708  0.96
2000 Zaman SH, Parent A, Laskey A, Lee MK, Borchelt DR, Sisodia SS, Malinow R. Enhanced synaptic potentiation in transgenic mice expressing presenilin 1 familial Alzheimer's disease mutation is normalized with a benzodiazepine. Neurobiology of Disease. 7: 54-63. PMID 10671322 DOI: 10.1006/nbdi.1999.0271  0.96
2000 Price DL, Sisodia SS, Wong PC, Borchelt DR. Alzheimer's disease: The value of transgenic and gene targeted models Neuroscience News. 3: 71-81.  0.96
1999 Schilling G, Wood JD, Duan K, Slunt HH, Gonzales V, Yamada M, Cooper JK, Margolis RL, Jenkins NA, Copeland NG, Takahashi H, Tsuji S, Price DL, Borchelt DR, Ross CA. Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA. Neuron. 24: 275-86. PMID 10677044 DOI: 10.1016/S0896-6273(00)80839-9  0.96
1999 Ross CA, Wood JD, Schilling G, Peters MF, Nucifora FC, Cooper JK, Sharp AH, Margolis RL, Borchelt DR. Polyglutamine pathogenesis. Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 354: 1005-11. PMID 10434299 DOI: 10.1098/rstb.1999.0452  0.96
1999 Ratovitski T, Corson LB, Strain J, Wong P, Cleveland DW, Culotta VC, Borchelt DR. 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. 8: 1451-60. PMID 10400992 DOI: 10.1093/hmg/8.8.1451  0.96
1999 Iadecola C, Zhang F, Niwa K, Eckman C, Turner SK, Fischer E, Younkin S, Borchelt DR, Hsiao KK, Carlson GA. SOD1 rescues cerebral endothelial dysfunction in mice overexpressing amyloid precursor protein. Nature Neuroscience. 2: 157-61. PMID 10195200 DOI: 10.1038/5715  0.96
1999 Parent A, Linden DJ, Sisodia SS, Borchelt DR. Synaptic transmission and hippocampal long-term potentiation in transgenic mice expressing FAD-linked presenilin 1. Neurobiology of Disease. 6: 56-62. PMID 10078973 DOI: 10.1006/nbdi.1998.0207  0.96
1999 Schilling G, Becher MW, Sharp AH, Jinnah HA, Duan K, Kotzuk JA, Slunt HH, Ratovitski T, Cooper JK, Jenkins NA, Copeland NG, Price DL, Ross CA, Borchelt DR. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Human Molecular Genetics. 8: 397-407. PMID 9949199  0.96
1999 Schilling G, Becher MW, Sharp AH, Jinnah HA, Duan K, Kotzuk JA, Slunt HH, Ratovitski T, Cooper JK, Jenkins NA, Copeland NG, Price DL, Ross CA, Borchelt DR. Corrigendum: Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin (Human Molecular Genetics (1999) 8 (397-407)) Human Molecular Genetics. 8: 943.  0.96
1998 Borchelt DR. Inherited neurodegenerative diseases and transgenic models. Laboratory Animal Science. 48: 604-10. PMID 10090084  0.96
1998 Wong PC, Borchelt DR, Lee MK, Pardo CA, Thinakaran G, Martin LJ, Sisodia SS, Price DL. Familial amyotrophic lateral sclerosis and Alzheimer's disease. Transgenic models. Advances in Experimental Medicine and Biology. 446: 145-59. PMID 10079842  0.96
1998 Price DL, Tanzi RE, Borchelt DR, Sisodia SS. Alzheimer's disease: genetic studies and transgenic models. Annual Review of Genetics. 32: 461-93. PMID 9928488 DOI: 10.1146/annurev.genet.32.1.461  0.96
1998 Pasinelli P, Borchelt DR, Houseweart MK, Cleveland DW, Brown RH. 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. 95: 15763-8. PMID 9861044 DOI: 10.1073/pnas.95.26.15763  0.96
1998 Naruse S, Thinakaran G, Luo JJ, Kusiak JW, Tomita T, Iwatsubo T, Qian X, Ginty DD, Price DL, Borchelt DR, Wong PC, Sisodia SS. Effects of PS1 deficiency on membrane protein trafficking in neurons. Neuron. 21: 1213-21. PMID 9856475 DOI: 10.1016/S0896-6273(00)80637-6  0.96
1998 Price DL, Sisodia SS, Borchelt DR. Genetic neurodegenerative diseases: the human illness and transgenic models. Science (New York, N.Y.). 282: 1079-83. PMID 9804539  0.96
1998 Borchelt DR, Wong PC, Sisodia SS, Price DL. Transgenic mouse models of Alzheimer's disease and amyotrophic lateral sclerosis. Brain Pathology (Zurich, Switzerland). 8: 735-57. PMID 9804381  0.96
1998 Borchelt DR, Wong PC, Becher MW, Pardo CA, Lee MK, Xu ZS, Thinakaran G, Jenkins NA, Copeland NG, Sisodia SS, Cleveland DW, Price DL, Hoffman PN. Axonal transport of mutant superoxide dismutase 1 and focal axonal abnormalities in the proximal axons of transgenic mice. Neurobiology of Disease. 5: 27-35. PMID 9702785 DOI: 10.1006/nbdi.1998.0178  0.96
1998 Price DL, Sisodia SS, Borchelt DR. Alzheimer disease--when and why? Nature Genetics. 19: 314-6. PMID 9697686 DOI: 10.1038/1196  1
1998 Thinakaran G, Regard JB, Bouton CM, Harris CL, Price DL, Borchelt DR, Sisodia SS. Stable association of presenilin derivatives and absence of presenilin interactions with APP. Neurobiology of Disease. 4: 438-53. PMID 9666482 DOI: 10.1006/nbdi.1998.0171  0.96
1998 Borchelt DR. Metabolism of presenilin 1: influence of presenilin 1 on amyloid precursor protein processing. Neurobiology of Aging. 19: S15-8. PMID 9562461 DOI: 10.1016/S0197-4580(98)00026-8  0.96
1998 Davis JA, Naruse S, Chen H, Eckman C, Younkin S, Price DL, Borchelt DR, Sisodia SS, Wong PC. An Alzheimer's disease-linked PS1 variant rescues the developmental abnormalities of PS1-deficient embryos. Neuron. 20: 603-9. PMID 9539132 DOI: 10.1016/S0896-6273(00)80998-8  0.96
1998 Cooper JK, Schilling G, Peters MF, Herring WJ, Sharp AH, Kaminsky Z, Masone J, Khan FA, Delanoy M, Borchelt DR, Dawson VL, Dawson TM, Ross CA. Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. Human Molecular Genetics. 7: 783-90. PMID 9536081 DOI: 10.1093/hmg/7.5.783  0.96
1997 Price DL, Wong PC, Borchelt DR, Pardo CA, Thinakaran G, Doan AP, Lee MK, Martin LJ, Sisodia SS. Amyotrophic lateral sclerosis and Alzheimer disease. Lessons from model systems. Revue Neurologique. 153: 484-95. PMID 9683997  0.96
1997 Borchelt DR, Ratovitski T, van Lare J, Lee MK, Gonzales V, Jenkins NA, Copeland NG, Price DL, Sisodia SS. Accelerated amyloid deposition in the brains of transgenic mice coexpressing mutant presenilin 1 and amyloid precursor proteins. Neuron. 19: 939-45. PMID 9354339 DOI: 10.1016/S0896-6273(00)80974-5  0.96
1997 Thinakaran G, Harris CL, Ratovitski T, Davenport F, Slunt HH, Price DL, Borchelt DR, Sisodia SS. Evidence that levels of presenilins (PS1 and PS2) are coordinately regulated by competition for limiting cellular factors. The Journal of Biological Chemistry. 272: 28415-22. PMID 9353300 DOI: 10.1074/jbc.272.45.28415  0.96
1997 Ratovitski T, Slunt HH, Thinakaran G, Price DL, Sisodia SS, Borchelt DR. Endoproteolytic processing and stabilization of wild-type and mutant presenilin. The Journal of Biological Chemistry. 272: 24536-41. PMID 9305918 DOI: 10.1074/jbc.272.39.24536  0.96
1997 Carlson GA, Borchelt DR, Dake A, Turner S, Danielson V, Coffin JD, Eckman C, Meiners J, Nilsen SP, Younkin SG, Hsiao KK. Genetic modification of the phenotypes produced by amyloid precursor protein overexpression in transgenic mice. Human Molecular Genetics. 6: 1951-9. PMID 9302276 DOI: 10.1093/hmg/6.11.1951  0.96
1997 Lee MK, Borchelt DR, Kim G, Thinakaran G, Slunt HH, Ratovitski T, Martin LJ, Kittur A, Gandy S, Levey AI, Jenkins N, Copeland N, Price DL, Sisodia SS. Hyperaccumulation of FAD-linked presenilin 1 variants in vivo. Nature Medicine. 3: 756-60. PMID 9212102 DOI: 10.1038/nm0797-756  0.96
1997 Bruijn LI, Becher MW, Lee MK, Anderson KL, Jenkins NA, Copeland NG, Sisodia SS, Rothstein JD, Borchelt DR, Price DL, Cleveland DW. ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. Neuron. 18: 327-38. PMID 9052802 DOI: 10.1016/S0896-6273(00)80272-X  0.96
1996 Price DL, Borchelt DR, Wong PC, Pardo CA, Thinakaran G, Lee MK, Cleveland DW, Sisodia SS. Neurodegenerative diseases and model systems. Cold Spring Harbor Symposia On Quantitative Biology. 61: 725-38. PMID 9246498  0.96
1996 Borchelt DR, Davis J, Fischer M, Lee MK, Slunt HH, Ratovitsky T, Regard J, Copeland NG, Jenkins NA, Sisodia SS, Price DL. A vector for expressing foreign genes in the brains and hearts of transgenic mice. Genetic Analysis : Biomolecular Engineering. 13: 159-63. PMID 9117892 DOI: 10.1016/S1050-3862(96)00167-2  0.96
1996 Price DL, Becher MW, Wong PC, Borchelt DR, Lee MK, Sisodia SS. Inherited neurodegenerative diseases and transgenic models. Brain Pathology (Zurich, Switzerland). 6: 467-80. PMID 8944317  0.96
1996 Doan A, Thinakaran G, Borchelt DR, Slunt HH, Ratovitsky T, Podlisny M, Selkoe DJ, Seeger M, Gandy SE, Price DL, Sisodia SS. Protein topology of presenilin 1. Neuron. 17: 1023-30. PMID 8938133 DOI: 10.1016/S0896-6273(00)80232-9  0.96
1996 Borchelt DR, Thinakaran G, Eckman CB, Lee MK, Davenport F, Ratovitsky T, Prada CM, Kim G, Seekins S, Yager D, Slunt HH, Wang R, Seeger M, Levey AI, Gandy SE, et al. Familial Alzheimer's disease-linked presenilin 1 variants elevate Abeta1-42/1-40 ratio in vitro and in vivo. Neuron. 17: 1005-13. PMID 8938131 DOI: 10.1016/S0896-6273(00)80230-5  0.96
1996 Lee MK, Borchelt DR, Wong PC, Sisodia SS, Price DL. Transgenic models of neurodegenerative diseases. Current Opinion in Neurobiology. 6: 651-60. PMID 8937830 DOI: 10.1016/S0959-4388(96)80099-7  0.96
1996 Price DL, Koliatsos VE, Wong PC, Pardo CA, Borchelt DR, Lee MK, Cleveland DW, Griffin JW, Hoffman PN, Cork LC, Sisodia SS. Motor neuron disease and model systems: aetiologies, mechanisms and therapies. Ciba Foundation Symposium. 196: 3-13; discussion 13-. PMID 8866125  0.96
1996 Cleveland DW, Bruijn LI, Wong PC, Marszalek JR, Vechio JD, Lee MK, Xu XS, Borchelt DR, Sisodia SS, Price DL. Mechanisms of selective motor neuron death in transgenic mouse models of motor neuron disease. Neurology. 47: S54-61; discussion S. PMID 8858052  0.96
1996 Borchelt DR, Sisodia SS. Loss of functional prion protein: a role in prion disorders? Chemistry & Biology. 3: 619-21. PMID 8807894 DOI: 10.1016/S1074-5521(96)90128-3  0.96
1996 Thinakaran G, Borchelt DR, Lee MK, Slunt HH, Spitzer L, Kim G, Ratovitsky T, Davenport F, Nordstedt C, Seeger M, Hardy J, Levey AI, Gandy SE, Jenkins NA, Copeland NG, et al. Endoproteolysis of presenilin 1 and accumulation of processed derivatives in vivo. Neuron. 17: 181-90. PMID 8755489 DOI: 10.1016/S0896-6273(00)80291-3  0.96
1996 Wong PC, Borchelt DR, Lee MK, Pardo CA, Sisodia SS, Cleveland DW, Koliatsos VE, Price DL. Transgenic and gene-targeting approaches to model disorders of motor neurons Seminars in Neuroscience. 8: 163-169. DOI: 10.1006/smns.1996.0021  0.96
1995 Pardo CA, Xu Z, Borchelt DR, Price DL, Sisodia SS, Cleveland DW. 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. 92: 954-8. PMID 7862672 DOI: 10.1073/pnas.92.4.954  0.96
1995 Borchelt DR, Guarnieri M, Wong PC, Lee MK, Slunt HS, Xu ZS, Sisodia SS, Price DL, Cleveland DW. Superoxide dismutase 1 subunits with mutations linked to familial amyotrophic lateral sclerosis do not affect wild-type subunit function. The Journal of Biological Chemistry. 270: 3234-8. PMID 7852409 DOI: 10.1074/jbc.270.7.3234  0.96
1995 Sisodia SS, Martin LJ, Walker LC, Borchelt DR, Price DL. Cellular and molecular biology of Alzheimer's disease and animal models. Neuroimaging Clinics of North America. 5: 59-68. PMID 7743085  0.96
1995 Rabizadeh S, Gralla EB, Borchelt DR, Gwinn R, Valentine JS, Sisodia S, Wong P, Lee M, Hahn H, Bredesen DE. 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. 92: 3024-8. PMID 7708768 DOI: 10.1073/pnas.92.7.3024  0.96
1995 Wong PC, Pardo CA, Borchelt DR, Lee MK, Copeland NG, Jenkins NA, Sisodia SS, Cleveland DW, Price DL. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron. 14: 1105-16. PMID 7605627 DOI: 10.1016/0896-6273(95)90259-7  0.96
1995 Wong PC, Borchelt DR. Motor neuron disease caused by mutations in superoxide dismutase 1. Current Opinion in Neurology. 8: 294-301. PMID 7582045  0.96
1995 Hsiao KK, Borchelt DR, Olson K, Johannsdottir R, Kitt C, Yunis W, Xu S, Eckman C, Younkin S, Price D. Age-related CNS disorder and early death in transgenic FVB/N mice overexpressing Alzheimer amyloid precursor proteins. Neuron. 15: 1203-18. PMID 7576662 DOI: 10.1016/0896-6273(95)90107-8  0.96
1994 Borchelt DR, Lee MK, Slunt HS, Guarnieri M, Xu ZS, Wong PC, Brown RH, Price DL, Sisodia SS, Cleveland DW. 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. 91: 8292-6. PMID 8058797 DOI: 10.1073/pnas.91.17.8292  0.96
1994 Borchelt DR, Koliatsos VE, Guarnieri M, Pardo CA, Sisodia SS, Price DL. Rapid anterograde axonal transport of the cellular prion glycoprotein in the peripheral and central nervous systems. The Journal of Biological Chemistry. 269: 14711-4. PMID 7514179  0.96
1993 Price DL, Borchelt DR, Sisodia SS. 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. 90: 6381-4. PMID 8101988  0.96
1993 Borchelt DR, Rogers M, Stahl N, Telling G, Prusiner SB. Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchor. Glycobiology. 3: 319-29. PMID 7691278 DOI: 10.1093/glycob/3.4.319  0.96
1992 Price DL, Borchelt DR, Walker LC, Sisodia SS. Toxicity of synthetic A beta peptides and modeling of Alzheimer's disease. Neurobiology of Aging. 13: 623-5. PMID 1461354 DOI: 10.1016/0197-4580(92)90069-A  0.96
1992 Taraboulos A, Raeber AJ, Borchelt DR, Serban D, Prusiner SB. Synthesis and trafficking of prion proteins in cultured cells. Molecular Biology of the Cell. 3: 851-63. PMID 1356522  0.96
1992 Raeber AJ, Borchelt DR, Scott M, Prusiner SB. Attempts to convert the cellular prion protein into the scrapie isoform in cell-free systems. Journal of Virology. 66: 6155-63. PMID 1356161  0.96
1992 Borchelt DR, Taraboulos A, Prusiner SB. Evidence for synthesis of scrapie prion proteins in the endocytic pathway. The Journal of Biological Chemistry. 267: 16188-99. PMID 1353761  0.96
1990 Taraboulos A, Rogers M, Borchelt DR, McKinley MP, Scott M, Serban D, Prusiner SB. 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. 87: 8262-6. PMID 1978322  0.96
1990 Stahl N, Borchelt DR, Prusiner SB. Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C. Biochemistry. 29: 5405-12. PMID 1974460  0.96
1990 Borchelt DR, Scott M, Taraboulos A, Stahl N, Prusiner SB. Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells. The Journal of Cell Biology. 110: 743-52. PMID 1968466  0.96
1989 Borchelt DR, Perdue ML. Inhibition of avian retrovirus protein synthesis in the presence of host cellular mRNA. Archives of Virology. 107: 261-71. PMID 2554857 DOI: 10.1007/BF01317922  0.96
1989 Haraguchi T, Fisher S, Olofsson S, Endo T, Groth D, Tarentino A, Borchelt DR, Teplow D, Hood L, Burlingame A. Asparagine-linked glycosylation of the scrapie and cellular prion proteins. Archives of Biochemistry and Biophysics. 274: 1-13. PMID 2505674 DOI: 10.1016/0003-9861(89)90409-8  0.96
1988 Butler DA, Scott MR, Bockman JM, Borchelt DR, Taraboulos A, Hsiao KK, Kingsbury DT, Prusiner SB. Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins. Journal of Virology. 62: 1558-64. PMID 3282080  0.96
1987 Stahl N, Borchelt DR, Hsiao K, Prusiner SB. Scrapie prion protein contains a phosphatidylinositol glycolipid. Cell. 51: 229-40. PMID 2444340 DOI: 10.1016/0092-8674(87)90150-4  0.96
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