{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T13:41:39Z","timestamp":1791207699186,"version":"4.1.0"},"reference-count":98,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2002,7,1]],"date-time":"2002-07-01T00:00:00Z","timestamp":1025481600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nat Neurosci"],"published-print":{"date-parts":[[2002,7]]},"DOI":"10.1038\/nn0702-633","type":"journal-article","created":{"date-parts":[[2002,7,28]],"date-time":"2002-07-28T17:36:01Z","timestamp":1027877761000},"page":"633-639","source":"Crossref","is-referenced-by-count":200,"title":["Genetically engineered mouse models of neurodegenerative diseases"],"prefix":"10.1038","volume":"5","author":[{"given":"Philip C.","family":"Wong","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huaibin","family":"Cai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David R.","family":"Borchelt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donald L.","family":"Price","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","reference":[{"key":"BFnn0702633_CR1","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1146\/annurev.genet.32.1.461","volume":"32","author":"DL Price","year":"1998","unstructured":"Price, D.L., Tanzi, R.E., Borchelt, D.R. & Sisodia, S.S. Alzheimer's disease: genetic studies and transgenic models. Annu. Rev. Genet. 32, 461\u2013493 (1998).","journal-title":"Annu. Rev. Genet."},{"key":"BFnn0702633_CR2","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1016\/S0896-6273(00)81106-X","volume":"24","author":"VMY Lee","year":"1999","unstructured":"Lee, V.M.Y. & Trojanowski, J.Q. Neurodegenerative taupathies: human disease and transgenic mouse models. Neuron 24, 507\u2013510 (1999).","journal-title":"Neuron"},{"key":"BFnn0702633_CR3","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1016\/S0896-6273(00)81104-6","volume":"24","author":"X Lin","year":"1999","unstructured":"Lin, X., Cummings, C.J. & Zoghbi, H.Y. Expanding our understanding of polyglutamine diseases through mouse models. Neuron 24, 499\u2013502 (1999).","journal-title":"Neuron"},{"key":"BFnn0702633_CR4","doi-asserted-by":"crossref","first-page":"A32","DOI":"10.1038\/399a032","volume":"399","author":"SB Dunnett","year":"1999","unstructured":"Dunnett, S.B. & Bjorklund, A. Prospects for new restorative and neuroprotective treatments in Parkinson's Disease. Nature 399, A32\u2013A39 (1999).","journal-title":"Nature"},{"key":"BFnn0702633_CR5","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/S0092-8674(00)80623-6","volume":"101","author":"A Yamamoto","year":"2000","unstructured":"Yamamoto, A., Lucas, J.J. & Hen, R. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 101, 57\u201366 (2000).","journal-title":"Cell"},{"key":"BFnn0702633_CR6","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1126\/science.282.5391.1075","volume":"282","author":"J Hardy","year":"1998","unstructured":"Hardy, J. & Gwinn-Hardy, K. Genetic classification of primary neurodegenerative disease. Science 282, 1075\u20131079 (1998).","journal-title":"Science"},{"key":"BFnn0702633_CR7","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1152\/physrev.2001.81.2.741","volume":"81","author":"DJ Selkoe","year":"2001","unstructured":"Selkoe, D.J. Alzheimer's disease: genes, proteins and therapy. Physiol. Rev. 81, 741\u2013766 (2001).","journal-title":"Physiol. Rev."},{"key":"BFnn0702633_CR8","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1126\/science.282.5391.1079","volume":"282","author":"DL Price","year":"1998","unstructured":"Price, D.L., Sisodia, S.S. & Borchelt, D.R. Genetic neurodegenerative diseases: the human illness and transgenic models. Science 282, 1079\u20131083 (1998).","journal-title":"Science"},{"key":"BFnn0702633_CR9","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1093\/hmg\/8.3.397","volume":"8","author":"G Schilling","year":"1999","unstructured":"Schilling, G. et al. Intranuclear inclusions and neuritic pathology in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum. Mol. Genet. 8, 397\u2013407 (1999).","journal-title":"Hum. Mol. Genet."},{"key":"BFnn0702633_CR10","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1016\/S0959-4388(98)80090-1","volume":"8","author":"M Goedert","year":"1998","unstructured":"Goedert, M., Spillantini, M.G. & Davies, S.W. Filamentous nerve cell inclusions in neurodegenerative diseases. Curr. Opin. Neurobiol. 8, 619\u2013632 (1998).","journal-title":"Curr. Opin. Neurobiol."},{"key":"BFnn0702633_CR11","doi-asserted-by":"crossref","first-page":"566","DOI":"10.1016\/S0959-4388(99)00013-6","volume":"9","author":"HY Zoghbi","year":"1999","unstructured":"Zoghbi, H.Y. & Orr, H.T. Polyglutamine diseases: protein cleavage and aggregation. Curr. Opin. Neurobiol. 9, 566\u2013570 (1999).","journal-title":"Curr. Opin. Neurobiol."},{"key":"BFnn0702633_CR12","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1212\/WNL.55.2.166","volume":"55","author":"MS Albert","year":"2000","unstructured":"Albert, M.S. & Drachman, D.A. Alzheimer's disease. What is it, how many people have it, and why do we need to know? Neurology 55, 166\u2013168 (2000).","journal-title":"Neurology"},{"key":"BFnn0702633_CR13","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1016\/S0896-6273(00)81109-5","volume":"24","author":"MM Mesulam","year":"1999","unstructured":"Mesulam, M.M. Neuroplasticity failure in Alzheimer's disease: bridging the gape between plaques and tangles. Neuron 24, 521\u2013529 (1999).","journal-title":"Neuron"},{"key":"BFnn0702633_CR14","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1146\/annurev.neuro.21.1.479","volume":"21","author":"DL Price","year":"1998","unstructured":"Price, D.L. & Sisodia, S.S. Mutant genes in familial Alzheimer's disease and transgenic models. Annu. Rev. Neurosci. 21, 479\u2013505 (1998).","journal-title":"Annu. Rev. Neurosci."},{"key":"BFnn0702633_CR15","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1093\/jnen\/59.4.308","volume":"59","author":"TG Beach","year":"2000","unstructured":"Beach, T.G. et al. The cholinergic deficit coincides with A\u03b2 deposition at the earliest histopathologic stages of Alzheimer disease. J. Neuropathol. Exp. Neurol. 59, 308\u2013313 (2000).","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"BFnn0702633_CR16","doi-asserted-by":"crossref","first-page":"13269","DOI":"10.1021\/bi000637v","volume":"39","author":"LC Serpell","year":"2000","unstructured":"Serpell, L.C., Blake, C.C.F. & Fraser, P.E. Molecular structure of a fibrillar Alzheimer's A\u03b2 fragment. Biochemistry 39, 13269\u201313275 (2000).","journal-title":"Biochemistry"},{"key":"BFnn0702633_CR17","doi-asserted-by":"crossref","first-page":"3342","DOI":"10.1073\/pnas.96.7.3342","volume":"96","author":"PT Lansbury Jr.","year":"1999","unstructured":"Lansbury, P.T. Jr. Evolution of amyloid: what normal protein folding may tell us about fibrillogenesis and disease. Proc. Natl. Acad. Sci. USA 96, 3342\u20133344 (1999).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnn0702633_CR18","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/S0896-6273(01)00476-7","volume":"32","author":"RE Tanzi","year":"2001","unstructured":"Tanzi, R.E. New frontiers in Alzheimer's disease genetics. Neuron 32, 181\u2013184 (2001).","journal-title":"Neuron"},{"key":"BFnn0702633_CR19","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1016\/S0896-6273(01)00475-5","volume":"32","author":"DJ Selkoe","year":"2001","unstructured":"Selkoe, D.J. Clearing the brain's amyloid cobwebs. Neuron 32, 177\u2013180 (2001).","journal-title":"Neuron"},{"key":"BFnn0702633_CR20","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1038\/990114","volume":"402","author":"S Sinha","year":"1999","unstructured":"Sinha, S. et al. Purification and cloning of amyloid precursor protein \u03b2-secretase from human brain. Nature 402, 537\u2013540 (1999).","journal-title":"Nature"},{"key":"BFnn0702633_CR21","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1006\/mcne.1999.0811","volume":"14","author":"I Hussain","year":"1999","unstructured":"Hussain, I. et al. Identification of a novel aspartic protease (Asp 2) as \u03b2-secretase. Mol. Cell. Neurosci. 14, 419\u2013427 (1999).","journal-title":"Mol. Cell. Neurosci."},{"key":"BFnn0702633_CR22","doi-asserted-by":"crossref","first-page":"1456","DOI":"10.1073\/pnas.97.4.1456","volume":"97","author":"X Lin","year":"2000","unstructured":"Lin, X. et al. Human aspartic protease memapsin 2 cleaves the \u03b2-secretase site of \u03b2-amyloid precursor protein. Proc. Natl. Acad. Sci. USA 97, 1456\u20131460 (2000).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnn0702633_CR23","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1038\/990107","volume":"402","author":"R Yan","year":"1999","unstructured":"Yan, R. et al. Membrane-anchored aspartyl protease with Alzheimer's disease \u03b2-secretase activity. Nature 402, 533\u2013537 (1999).","journal-title":"Nature"},{"key":"BFnn0702633_CR24","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1126\/science.286.5440.735","volume":"286","author":"R Vassar","year":"1999","unstructured":"Vassar, R. et al. \u03b2-secretase cleavage of Alzheimer's amyloid precusor protein by the transmembrane aspartic protease BACE. Science 286, 735\u2013741 (1999).","journal-title":"Science"},{"key":"BFnn0702633_CR25","doi-asserted-by":"crossref","first-page":"1434","DOI":"10.1126\/science.293.5534.1434b","volume":"293","author":"PC Wong","year":"2001","unstructured":"Wong, P.C., Price, D.L. & Cai, H. The brain's susceptibility to amyloid plaques. Science 293, 1434\u20131435 (2001).","journal-title":"Science"},{"key":"BFnn0702633_CR26","doi-asserted-by":"crossref","first-page":"9712","DOI":"10.1073\/pnas.160115697","volume":"97","author":"M Farzan","year":"2000","unstructured":"Farzan, M., Schnitzler, C.E., Vasilieva, N., Leung, D. & Choe, H. BACE2, a \u03b2-secretase homolog, cleaves at the \u03b2 site and within the amyloid-\u03b2 region of the amyloid-\u03b2 precursor protein. Proc. Natl. Acad. Sci. USA 97, 9712\u20139717 (2000).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnn0702633_CR27","doi-asserted-by":"crossref","first-page":"887","DOI":"10.1038\/nn0901-887","volume":"4","author":"C Nilsberth","year":"2001","unstructured":"Nilsberth, C. et al. The 'Arctic' APP mutation (E693G) causes Alzheimer's disease by enhanced A\u03b2 protofibril formation. Nat. Neurosci. 4, 887\u2013893 (2001).","journal-title":"Nat. Neurosci."},{"key":"BFnn0702633_CR28","doi-asserted-by":"crossref","first-page":"754","DOI":"10.1038\/375754a0","volume":"375","author":"R Sherrington","year":"1995","unstructured":"Sherrington, R. et al. Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease. Nature 375, 754\u2013760 (1995).","journal-title":"Nature"},{"key":"BFnn0702633_CR29","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1126\/science.7638622","volume":"269","author":"E Levy-Lahad","year":"1995","unstructured":"Levy-Lahad, E. et al. Candidate gene for the chromosome 1 familial Alzheimer's disease locus. Science 269, 973\u2013977 (1995).","journal-title":"Science"},{"key":"BFnn0702633_CR30","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1038\/376775a0","volume":"376","author":"EI Rogaev","year":"1995","unstructured":"Rogaev, E.I. et al. Familial Alzheimer's disease in kindreds with missense mutations in a gene on chromosome 1 related to the Alzheimer's disease type 3 gene. Nature 376, 775\u2013778 (1995).","journal-title":"Nature"},{"key":"BFnn0702633_CR31","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/S0896-6273(00)80291-3","volume":"17","author":"G Thinakaran","year":"1996","unstructured":"Thinakaran, G. et al. Endoproteolysis of presenilin 1 and accumulation of processed derivatives in vivo. Neuron 17, 181\u2013190 (1996).","journal-title":"Neuron"},{"key":"BFnn0702633_CR32","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1038\/nrn785","volume":"3","author":"SS Sisodia","year":"2002","unstructured":"Sisodia, S.S. & George-Hyslop, P.H. gamma-secretase, Notch, A\u03b2 and Alzheimer's disease: where do the presenilins fit in? Nat. Rev. Neurosci. 3, 281\u2013290 (2002).","journal-title":"Nat. Rev. Neurosci."},{"key":"BFnn0702633_CR33","doi-asserted-by":"crossref","first-page":"1449","DOI":"10.1126\/science.1064638","volume":"293","author":"WP Esler","year":"2001","unstructured":"Esler, W.P. & Wolfe, M.S. A portrait of Alzheimer secretases\u2014new features and familiar faces. Science 293, 1449\u20131454 (2001).","journal-title":"Science"},{"key":"BFnn0702633_CR34","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1016\/S0896-6273(00)00051-9","volume":"27","author":"R Vassar","year":"2000","unstructured":"Vassar, R. & Citron, M. A\u03b2-generating enzymes: recent advances in \u03b2- and \u03b3-secretase research. Neuron 27, 419\u2013422 (2000).","journal-title":"Neuron"},{"key":"BFnn0702633_CR35","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1038\/34910","volume":"391","author":"B De Strooper","year":"1998","unstructured":"De Strooper, B. et al. Deficiency of presenilin-1 inhibits the normal cleavage of amyloid precursor protein. Nature 391, 387\u2013390 (1998).","journal-title":"Nature"},{"key":"BFnn0702633_CR36","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1038\/19077","volume":"398","author":"MS Wolfe","year":"1999","unstructured":"Wolfe, M.S. et al. Two transmembrane aspartates in presenilin-1 required for presenilin endoproteolysis and secretase activity. Nature 398, 513\u2013517 (1999).","journal-title":"Nature"},{"key":"BFnn0702633_CR37","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/S0896-6273(02)00585-8","volume":"33","author":"R Kopan","year":"2002","unstructured":"Kopan, R. & Goate, A. Aph-2\/Nicastrin: an essential component of gamma-secretase and regulator of notch signaling and presenilin localization. Neuron 33, 321\u2013324 (2002).","journal-title":"Neuron"},{"key":"BFnn0702633_CR38","doi-asserted-by":"crossref","first-page":"14088","DOI":"10.1073\/pnas.96.24.14088","volume":"96","author":"ME Calhoun","year":"1999","unstructured":"Calhoun, M.E. et al. Neuronal overexpression of mutant amyloid precursor protein results in prominent deposition of cerebrovascular amyloid. Proc. Natl. Acad. Sci. USA 96, 14088\u201314093 (1999).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnn0702633_CR39","doi-asserted-by":"crossref","first-page":"975","DOI":"10.1038\/35050103","volume":"408","author":"G Chen","year":"2000","unstructured":"Chen, G. et al. A learning deficit related to age and \u03b2-amyloid plaques in a mouse model of Alzheimer's disease. Nature 408, 975\u2013979 (2000).","journal-title":"Nature"},{"key":"BFnn0702633_CR40","doi-asserted-by":"crossref","first-page":"4050","DOI":"10.1523\/JNEUROSCI.20-11-04050.2000","volume":"20","author":"L Mucke","year":"2000","unstructured":"Mucke, L. et al. High-level neuronal expression of A\u03b21-42 in wild-type human amyloid protein precursor transgenic mice: synaptotoxicity without plaque formation. J. Neurosci. 20, 4050\u20134058 (2000).","journal-title":"J. Neurosci."},{"key":"BFnn0702633_CR41","doi-asserted-by":"crossref","first-page":"3228","DOI":"10.1073\/pnas.96.6.3228","volume":"96","author":"AY Hsia","year":"1999","unstructured":"Hsia, A.Y. et al. Plaque-independent disruption of neural circuits in Alzheimer's disease mouse models. Proc. Natl. Acad. Sci. USA 96, 3228\u20133233 (1999).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnn0702633_CR42","doi-asserted-by":"crossref","first-page":"1487","DOI":"10.1126\/science.1058189","volume":"293","author":"J Lewis","year":"2001","unstructured":"Lewis, J. et al. Enhanced neurofibrillary degeneration in transgenic mice expressing mutant tau and APP. Science 293, 1487\u20131491 (2001).","journal-title":"Science"},{"key":"BFnn0702633_CR43","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1074\/jbc.M006531200","volume":"276","author":"J G\u00f6tz","year":"2001","unstructured":"G\u00f6tz, J., Chen, F., Barmettler, R. & Nitsch, R.M. Tau filament formation in transgenic mice expressing P301L tau. J. Biol. Chem. 276, 529\u2013534 (2001).","journal-title":"J. Biol. Chem."},{"key":"BFnn0702633_CR44","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1016\/S0896-6273(00)80974-5","volume":"19","author":"DR Borchelt","year":"1997","unstructured":"Borchelt, D.R. et al. Accelerated amyloid deposition in the brains of transgenic mice co-expressing mutant presenilin 1 and amyloid precursor proteins. Neuron 19, 939\u2013945 (1997).","journal-title":"Neuron"},{"key":"BFnn0702633_CR45","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1016\/0092-8674(95)90073-X","volume":"81","author":"H Zheng","year":"1995","unstructured":"Zheng, H. et al. \u03b2-amyloid precursor protein-deficient mice show reactive gliosis and decreased locomotor activity. Cell 81, 525\u2013531 (1995).","journal-title":"Cell"},{"key":"BFnn0702633_CR46","doi-asserted-by":"crossref","first-page":"7951","DOI":"10.1523\/JNEUROSCI.20-21-07951.2000","volume":"20","author":"S Heber","year":"2000","unstructured":"Heber, S. et al. Mice with combined gene knock-outs reveal essential and partially redundant functions of amyloid precursor protein family members. J. Neurosci. 20, 7951\u20137963 (2000).","journal-title":"J. Neurosci."},{"key":"BFnn0702633_CR47","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1038\/19091","volume":"398","author":"G Struhl","year":"1999","unstructured":"Struhl, G. & Greenwald, I. Presenilin is required for activity and nuclear access of notch in drosophila. Nature 398, 522\u2013525 (1999).","journal-title":"Nature"},{"key":"BFnn0702633_CR48","doi-asserted-by":"publisher","first-page":"288","DOI":"10.1038\/387288a0","volume":"387","author":"PC Wong","year":"1997","unstructured":"Wong, P.C. et al. Presenilin 1 is required for Notch1 and Dll1 expression in the paraxial mesoderm. Nature 387, 288\u2013292 (1997).","journal-title":"Nature"},{"key":"BFnn0702633_CR49","doi-asserted-by":"publisher","first-page":"2801","DOI":"10.1101\/gad.13.21.2801","volume":"13","author":"D Donoviel","year":"1999","unstructured":"Donoviel, D. et al. Mice lacking both presenilin genes exhibit early embryonic patterning defects. Genes Dev. 13, 2801\u20132810 (1999).","journal-title":"Genes Dev."},{"key":"BFnn0702633_CR50","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1016\/S0896-6273(01)00417-2","volume":"31","author":"H Yu","year":"2001","unstructured":"Yu, H. et al. APP processing and synaptic plasticity in presenilin-1 conditional knockout mice. Neuron 31, 713\u2013726 (2001).","journal-title":"Neuron"},{"key":"BFnn0702633_CR51","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1016\/S0896-6273(01)00523-2","volume":"32","author":"R Feng","year":"2001","unstructured":"Feng, R. et al. Deficient neurogenesis in forebrain-specific presenilin-1 knockout mice is associated with reduced clearance of hippocampal memory traces. Neuron 32, 911\u2013926 (2001).","journal-title":"Neuron"},{"key":"BFnn0702633_CR52","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1038\/85064","volume":"4","author":"H Cai","year":"2001","unstructured":"Cai, H. et al. BACE1 is the major \u03b2-secretase for generation of A\u03b2 peptides by neurons. Nat. Neurosci. 4, 233\u2013234 (2001).","journal-title":"Nat. Neurosci."},{"key":"BFnn0702633_CR53","first-page":"231","volume":"4","author":"Y Luo","year":"2001","unstructured":"Luo, Y. et al. Mice deficient in BACE1, the Alzheimer's \u03b2-secretase, have normal phenotype and abolished \u03b2-amyloid generation. Nature 4, 231\u2013232 (2001).","journal-title":"Nature"},{"key":"BFnn0702633_CR54","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1093\/hmg\/10.12.1317","volume":"10","author":"SL Roberds","year":"2001","unstructured":"Roberds, S.L. et al. BACE knockout mice are healthy despite lacking the primary \u03b2-secretase activity in brain: implication for Alzheimer's disease therapeutics. Hum. Mol. Genet. 10, 1317\u20131324 (2001).","journal-title":"Hum. Mol. Genet."},{"key":"BFnn0702633_CR55","doi-asserted-by":"crossref","first-page":"12019","DOI":"10.1074\/jbc.M008861200","volume":"276","author":"U Bodendorf","year":"2001","unstructured":"Bodendorf, U., Fischer, F., Bodian, D., Multhaup, G. & Paganetti, P. A splice variant of \u03b2-secretase deficient in the amyloidogenic processing of the amyloid precursor protein. J. Biol. Chem. 276, 12019\u201312023 (2001).","journal-title":"J. Biol. Chem."},{"key":"BFnn0702633_CR56","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1038\/35015085","volume":"405","author":"YM Li","year":"2000","unstructured":"Li, Y.M. et al. Photoactivated \u03b3-secretase inhibitors directed to the active site convalently label presenilin 1. Nature 405, 689\u2013693 (2000).","journal-title":"Nature"},{"key":"BFnn0702633_CR57","doi-asserted-by":"crossref","first-page":"966","DOI":"10.1038\/35016111","volume":"405","author":"SS Huppert","year":"2000","unstructured":"Huppert, S.S. et al. Embryonic lethality in mice homozygous for a processing-deficient allele of Notch 1. Nature 405, 966\u2013970 (2000).","journal-title":"Nature"},{"key":"BFnn0702633_CR58","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1038\/35074581","volume":"3","author":"A Petit","year":"2001","unstructured":"Petit, A. et al. New protease inhibitors prevent \u03b3-secretase-mediated production of A\u03b240\/42 without affecting Notch clevage. Nat. Cell Biol. 3, 507\u2013511 (2001).","journal-title":"Nat. Cell Biol."},{"key":"BFnn0702633_CR59","doi-asserted-by":"crossref","first-page":"7487","DOI":"10.1073\/pnas.131202798","volume":"98","author":"BK Hadland","year":"2001","unstructured":"Hadland, B.K. et al. \u03b3-secretase inhibitors repress thymocyte development. Proc. Natl. Acad. Sci. USA 98, 7487\u20137491 (2001).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnn0702633_CR60","doi-asserted-by":"crossref","first-page":"916","DOI":"10.1038\/78682","volume":"6","author":"F Bard","year":"2000","unstructured":"Bard, F. et al. Peripherally administered antibodies against amyloid \u03b2-peptide enter the central nervous system and reduce pathology in a mouse model of Alzheimer's disease. Nat. Med. 6, 916\u2013919 (2000).","journal-title":"Nat. Med."},{"key":"BFnn0702633_CR61","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1038\/22124","volume":"400","author":"D Schenk","year":"1999","unstructured":"Schenk, D. et al. Immunization with amyloid attenuates Alzheimer-disease-like pathology in the PDAPP mouse. Nature 400, 173\u2013177 (1999).","journal-title":"Nature"},{"key":"BFnn0702633_CR62","doi-asserted-by":"crossref","first-page":"8850","DOI":"10.1073\/pnas.151261398","volume":"98","author":"RB DeMattos","year":"2001","unstructured":"DeMattos, R.B. et al. Peripheral anti-A\u03b2 antibody alters CNS and plasma A\u03b2 clearance and decreases brain A\u03b2 burden in a mouse model of Alzheimer's disease. Proc. Natl. Acad. Sci. USA 98, 8850\u20138855 (2001).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnn0702633_CR63","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1038\/35050110","volume":"408","author":"C Janus","year":"2000","unstructured":"Janus, C. et al. A\u03b2 peptide immunization reduces behavioural impairment and plaques in a model of Alzheimer's disease. Nature 408, 979\u2013982 (2000).","journal-title":"Nature"},{"key":"BFnn0702633_CR64","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1038\/35050116","volume":"408","author":"D Morgan","year":"2000","unstructured":"Morgan, D. et al. A \u03b2 peptide vaccination prevents memory loss in an animal model of Alzheimer's disease. Nature 408, 982\u2013985 (2000).","journal-title":"Nature"},{"key":"BFnn0702633_CR65","first-page":"83","volume-title":"Amyotrophic Lateral Sclerosis","author":"PG Ince","year":"2000","unstructured":"Ince, P.G. in Amyotrophic Lateral Sclerosis (eds. Brown, R. H. Jr., Meininger, V. & Swash, M.) 83\u2013112 (Martin Dunitz, London, 2000)."},{"key":"BFnn0702633_CR66","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1097\/00005072-199905000-00005","volume":"58","author":"LJ Martin","year":"1999","unstructured":"Martin, L.J. Neuronal death in amyotrophic lateral sclerosis is apoptosis: possible contribution of a programmed cell death mechanism. J. Neuropathol. Exp. Neurol. 58, 459\u2013471 (1999).","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"BFnn0702633_CR67","first-page":"223","volume-title":"Amyotrophic Lateral Sclerosis","author":"PM Andersen","year":"2000","unstructured":"Andersen, P.M., Morita, M. & Brown, R.H. Jr. in Amyotrophic Lateral Sclerosis (eds. Brown, R. H. Jr., Meininger, V. & Swash, M.) 223\u2013250 (Martin Dunitz, London, 2000)."},{"key":"BFnn0702633_CR68","first-page":"806","volume":"2","author":"DW Cleveland","year":"2001","unstructured":"Cleveland, D.W. & Rothstein, J.D. From Charcot to Lou Gehrig: deciphering selective motor neurons death in ALS. Nature 2, 806\u2013819 (2001).","journal-title":"Nature"},{"key":"BFnn0702633_CR69","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1038\/ng1001-166","volume":"29","author":"S Hadano","year":"2001","unstructured":"Hadano, S. et al. A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nat. Genet. 29, 166\u2013173 (2001).","journal-title":"Nat. Genet."},{"key":"BFnn0702633_CR70","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1038\/ng1001-160","volume":"29","author":"Y Yang","year":"2001","unstructured":"Yang, Y. et al. The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis. Nat. Genet. 29, 160\u2013165 (2001).","journal-title":"Nat. Genet."},{"key":"BFnn0702633_CR71","doi-asserted-by":"crossref","first-page":"1772","DOI":"10.1126\/science.8209258","volume":"264","author":"ME Gurney","year":"1994","unstructured":"Gurney, M.E. et al. Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation. Science 264, 1772\u20131775 (1994).","journal-title":"Science"},{"key":"BFnn0702633_CR72","doi-asserted-by":"crossref","first-page":"1105","DOI":"10.1016\/0896-6273(95)90259-7","volume":"14","author":"PC Wong","year":"1995","unstructured":"Wong, P.C. et al. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14, 1105\u20131116 (1995).","journal-title":"Neuron"},{"key":"BFnn0702633_CR73","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/S0896-6273(00)80272-X","volume":"18","author":"LI Bruijn","year":"1997","unstructured":"Bruijn, L.I. et al. ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. Neuron 18, 327\u2013338 (1997).","journal-title":"Neuron"},{"key":"BFnn0702633_CR74","first-page":"263","volume-title":"Amyotrophic Lateral Sclerosis","author":"M Jackson","year":"2000","unstructured":"Jackson, M. & Rothstein, J.D. in Amyotrophic Lateral Sclerosis (eds. Brown, R. H. Jr., Meininger, V. & Swash, M.) 263\u2013278 (Martin Dunitz, London, 2000)."},{"key":"BFnn0702633_CR75","first-page":"113","volume-title":"Amyotrophic Lateral Sclerosis","author":"PJ Shaw","year":"2000","unstructured":"Shaw, P.J. in Amyotrophic Lateral Sclerosis (eds. Brown, R. H. Jr., Meininger, V. & Swash, M.) 113\u2013144 (Martin Dunitz, London, 2000)."},{"key":"BFnn0702633_CR76","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1038\/4553","volume":"2","author":"TL Williamson","year":"1999","unstructured":"Williamson, T.L. & Cleveland, D.W. Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons. Nat. Neurosci. 2, 50\u201356 (1999).","journal-title":"Nat. Neurosci."},{"key":"BFnn0702633_CR77","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1016\/S0092-8674(01)00244-6","volume":"104","author":"J-P Julien","year":"2001","unstructured":"Julien, J.-P. Amyotrophic lateral sclerosis: unfolding the toxicity of the misfolded. Cell 104, 581\u2013591 (2001).","journal-title":"Cell"},{"key":"BFnn0702633_CR78","first-page":"15763","volume":"95","author":"P Pasinelli","year":"1998","unstructured":"Pasinelli, P., Borchelt, D.R., Houseweart, M.K., Cleveland, D.W. & Brown, R.H. Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase. Neurobiology 95, 15763\u201315768 (1998).","journal-title":"Neurobiology"},{"key":"BFnn0702633_CR79","doi-asserted-by":"crossref","first-page":"9626","DOI":"10.1073\/pnas.95.16.9626","volume":"95","author":"S Couillard-Despr\u00e9s","year":"1998","unstructured":"Couillard-Despr\u00e9s, S. et al. Protective effect of neurofilament NF-H overexpression in motor neuron disease induced by mutant superoxide dismutase. Proc. Natl. Acad. Sci. USA 95, 9626\u20139630 (1998).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnn0702633_CR80","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1038\/6568","volume":"5","author":"P Klivenyi","year":"1999","unstructured":"Klivenyi, P. et al. Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis. Nat. Med. 5, 347\u2013350 (1999).","journal-title":"Nat. Med."},{"key":"BFnn0702633_CR81","doi-asserted-by":"crossref","first-page":"2498","DOI":"10.1126\/science.286.5449.2498","volume":"286","author":"AG Est\u00e9vez","year":"1999","unstructured":"Est\u00e9vez, A.G. et al. Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutase. Science 286, 2498\u20132500 (1999).","journal-title":"Science"},{"key":"BFnn0702633_CR82","doi-asserted-by":"crossref","first-page":"2886","DOI":"10.1073\/pnas.040461197","volume":"97","author":"PC Wong","year":"2000","unstructured":"Wong, P.C. et al. Copper chaperone for superoxide dismutase is essential to activate mammalian Cu\/Zn superoxide dismutase. Proc. Natl. Acad. Sci. USA 97, 2886\u20132891 (2000).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnn0702633_CR83","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1038\/nn823","volume":"5","author":"JR Subramaniam","year":"2002","unstructured":"Subramaniam, J.R. et al. Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading. Nat. Neurosci. 5, 301\u2013307 (2002).","journal-title":"Nat. Neurosci."},{"key":"BFnn0702633_CR84","doi-asserted-by":"crossref","first-page":"12571","DOI":"10.1073\/pnas.220417997","volume":"97","author":"JA Johnston","year":"2000","unstructured":"Johnston, J.A., Dalton, M.J., Gurney, M.E. & Kopito, R.R. Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis. Proc. Natl. Acad. Sci. USA 97, 12571\u201312576 (2000).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnn0702633_CR85","doi-asserted-by":"crossref","first-page":"1851","DOI":"10.1126\/science.281.5384.1851","volume":"281","author":"LI Bruijn","year":"1998","unstructured":"Bruijn, L.I. et al. Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science 281, 1851\u20131854 (1998).","journal-title":"Science"},{"key":"BFnn0702633_CR86","doi-asserted-by":"crossref","first-page":"5321","DOI":"10.1523\/JNEUROSCI.20-14-05321.2000","volume":"20","author":"JM Beaulieu","year":"2000","unstructured":"Beaulieu, J.M., Jacomy, H. & Julien, J.P. Formation of intermediate filament protein aggregates with disparate effects in two transgenic mouse models lacking the neurofilament light subunit. J. Neurosci. 20, 5321\u20135328 (2000).","journal-title":"J. Neurosci."},{"key":"BFnn0702633_CR87","doi-asserted-by":"crossref","first-page":"584","DOI":"10.1038\/35378","volume":"391","author":"J Eyer","year":"1998","unstructured":"Eyer, J., Cleveland, D.W., Wong, P.C. & Peterson, A.C. Pathogenesis of two axonopathies does not require axonal neurofilaments. Nature 391, 584\u2013587 (1998).","journal-title":"Nature"},{"key":"BFnn0702633_CR88","doi-asserted-by":"crossref","first-page":"9631","DOI":"10.1073\/pnas.95.16.9631","volume":"95","author":"TL Williamson","year":"1998","unstructured":"Williamson, T.L. et al. Absence of neurofilaments reduces the selective vulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant. Proc. Natl. Acad. Sci. USA 95, 9631\u20139636 (1998).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnn0702633_CR89","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/S0304-3940(00)00808-9","volume":"281","author":"J Kong","year":"2000","unstructured":"Kong, J. & Xu, Z. Overexpression of neurofilament subunit NF-L and NF-H extends survival of a mouse model for amyotrophic lateral sclerosis. Neurosci. Lett. 281, 72\u201374 (2000).","journal-title":"Neurosci. Lett."},{"key":"BFnn0702633_CR90","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1006\/nbdi.1996.0010","volume":"3","author":"T Crawford","year":"1996","unstructured":"Crawford, T. & Pardo, C.A. The neurobiology of childhood spinal muscular atrophy. Neurobiol. Dis. 3, 97\u2013110 (1996).","journal-title":"Neurobiol. Dis."},{"key":"BFnn0702633_CR91","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/0092-8674(95)90460-3","volume":"80","author":"S Lefebvre","year":"1995","unstructured":"Lefebvre, S. et al. Identification and characterization of a spinal muscular atrophy-determining gene. Cell 80, 155\u2013165 (1995).","journal-title":"Cell"},{"key":"BFnn0702633_CR92","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1016\/0092-8674(95)90461-1","volume":"80","author":"N Roy","year":"1995","unstructured":"Roy, N. et al. The gene for neuronal apoptosis inhibitory protein is partially deleted in individuals with spinal muscular atrophy. Cell 80, 167\u2013178 (1995).","journal-title":"Cell"},{"key":"BFnn0702633_CR93","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1016\/S0092-8674(00)80368-2","volume":"90","author":"U Fischer","year":"1997","unstructured":"Fischer, U., Liu, Q. & Dreyfuss, G. The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis. Cell 90, 1023\u20131029 (1997).","journal-title":"Cell"},{"key":"BFnn0702633_CR94","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1093\/hmg\/9.1.47","volume":"9","author":"S Pagliardini","year":"2000","unstructured":"Pagliardini, S. et al. Subcellular localization and axonal transport of the survival motor neuron (SMN) protein in the developing rat spinal cord. Hum. Mol. Genet. 9, 47\u201356 (2000).","journal-title":"Hum. Mol. Genet."},{"key":"BFnn0702633_CR95","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1093\/hmg\/9.3.341","volume":"9","author":"S Jablonka","year":"2000","unstructured":"Jablonka, S., Schrank, B., Kralewski, M., Rossoll, W. & Sendtner, M. Reduced survival motor neuron (Smn) gene dose in mice leads to motor neuron degeneration: an animal model for spinal muscular atrophy type III. Hum. Mol. Genet. 9, 341\u2013346 (2000).","journal-title":"Hum. Mol. Genet."},{"key":"BFnn0702633_CR96","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1038\/71709","volume":"24","author":"HM Hsieh-Li","year":"2000","unstructured":"Hsieh-Li, H.M. et al. A mouse model for spinal muscular atrophy. Nat. Genet. 24, 66\u201370 (2000).","journal-title":"Nat. Genet."},{"key":"BFnn0702633_CR97","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1093\/hmg\/9.3.333","volume":"9","author":"UR Monani","year":"2000","unstructured":"Monani, U.R. et al. The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn\u2212\/\u2212 mice and results in a mouse with spinal muscular atrophy. Hum. Mol. Genet. 9, 333\u2013339 (2000).","journal-title":"Hum. Mol. Genet."},{"key":"BFnn0702633_CR98","doi-asserted-by":"crossref","first-page":"9808","DOI":"10.1073\/pnas.171105098","volume":"98","author":"J-G Chang","year":"2001","unstructured":"Chang, J.-G. et al. Treatment of spinal muscular atrphy by sodium butyrate. Proc. Natl. Acad. Sci. USA 98, 9808\u20139813 (2001).","journal-title":"Proc. Natl. Acad. Sci. USA"}],"container-title":["Nature Neuroscience"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.nature.com\/articles\/nn0702-633.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/nn0702-633","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/nn0702-633.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,18]],"date-time":"2023-05-18T19:42:55Z","timestamp":1684438975000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/nn0702-633"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2002,7]]},"references-count":98,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2002,7]]}},"alternative-id":["BFnn0702633"],"URL":"https:\/\/doi.org\/10.1038\/nn0702-633","relation":{},"ISSN":["1097-6256","1546-1726"],"issn-type":[{"value":"1097-6256","type":"print"},{"value":"1546-1726","type":"electronic"}],"subject":[],"published":{"date-parts":[[2002,7]]}}}