title: Genetically engineered models relevant to neurodegenerative disorders: their value for understanding disease mechanisms and designing/testing experimental therapeutics.
abstract: Significant progress in the identification of disease-specific genes for a variety of neurodegenerative diseases have provided opportunities to understand molecular mechanisms and to test experimental therapeutic for these disorders. Recent works on clarifying the selective vulnerability of neurons and pathogenic mechanisms using genetically engineered mouse models of familial forms of Alzheimer's disease and motor neuron disease will be reviewed.
authors.affiliation: Department of Pathology The Johns Hopkins University School of Medicine, Baltimore, MD 21205-2196, USA.
authors.forename: P C
authors.initials: PC
authors.lastname: Wong
authors.forename: H
authors.initials: H
authors.lastname: Cai
authors.forename: D R
authors.initials: DR
authors.lastname: Borchelt
authors.forename: D L
authors.initials: DL
authors.lastname: Price
mesh_terms: D000544:Alzheimer Disease / Q000235:genetics* / Q000378:metabolism / Q000503:physiopathology; D053829:Amyloid Precursor Protein Secretases; D000818:Animals; D016282:Aspartic Acid Endopeptidases / Q000037:antagonists & inhibitors / Q000235:genetics / Q000378:metabolism; D004195:Disease Models, Animal; D010450:Endopeptidases; D005818:Genetic Engineering / Q000639:trends*; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic / Q000235:genetics* / Q000378:metabolism; D016472:Motor Neuron Disease / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology; D009154:Mutation / Q000235:genetics; D013482:Superoxide Dismutase / Q000037:antagonists & inhibitors / Q000235:genetics / Q000378:metabolism; D000072105:Superoxide Dismutase-1
chemical_list: C000606290:SOD1 protein, human; C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1; D053829:Amyloid Precursor Protein Secretases; D010450:Endopeptidases; D016282:Aspartic Acid Endopeptidases; C509280:BACE1 protein, human; C509282:Bace1 protein, mouse