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Early-onset Amyloid Deposition and Cognitive Deficits in Transgenic Mice Expressing a Double Mutant Form of Amyloid Precursor Protein 695*
We have created early-onset transgenic (Tg) models by exploiting the synergistic effects of familial Alzheimer's disease mutations on amyloid β-peptide (Aβ) biogenesis. TgCRND8 mice encode a doubleExpand
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Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel.
The novel locus Prnd is 16 kb downstream of the mouse prion protein (PrP) gene Prnp and encodes a 179 residue PrP-like protein designated doppel (Dpl). Prnd generates major transcripts of 1.7 and 2.7Expand
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Hypersensitivity of DJ-1-deficient mice to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyrindine (MPTP) and oxidative stress.
  • R. Kim, P. Smith, +12 authors T. Mak
  • Medicine, Biology
  • Proceedings of the National Academy of Sciences…
  • 5 April 2005
Mutations of the DJ-1 (PARK7) gene are linked to familial Parkinson's disease. We used gene targeting to generate DJ-1-deficient mice that were viable, fertile, and showed no gross anatomical orExpand
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  • Open Access
A cellular gene encodes scrapie PrP 27-30 protein
A clone encoding PrP 27-30, the major protein in purified preparations of scrapie agent, was selected from a scrapie-infected hamster brain cDNA library by oligonucleotide probes corresponding to theExpand
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The CNS glycoprotein Shadoo has PrPC-like protective properties and displays reduced levels in prion infections
The cellular prion protein, PrPC, is neuroprotective in a number of settings and in particular prevents cerebellar degeneration mediated by CNS‐expressed Doppel or internally deleted PrP (‘ΔPrP’).Expand
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  • Open Access
Linkage of a prion protein missense variant to Gerstmann–Sträussler syndrome
GERSTMANN–Sträussler syndrome is a rare familial neuro-degenerative condition that is vertically transmitted, in an apparently autosomal dominant way1. It can also be horizontally transmitted toExpand
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Complete genomic sequence and analysis of the prion protein gene region from three mammalian species.
The prion protein (PrP), first identified in scrapie-infected rodents, is encoded by a single exon of a single-copy chromosomal gene. In addition to the protein-coding exon, PrP genes in mammalsExpand
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TMP21 is a presenilin complex component that modulates gamma-secretase but not epsilon-secretase activity.
The presenilin proteins (PS1 and PS2) and their interacting partners nicastrin, aph-1 (refs 4, 5) and pen-2 (ref. 5) form a series of high-molecular-mass, membrane-bound protein complexes that areExpand
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Abeta 1-40-related reduction in functional hyperemia in mouse neocortex during somatosensory activation.
Peptides derived from proteolytic processing of the beta-amyloid precursor protein (APP), including the amyloid-beta peptide (Abeta), play a critical role in the pathogenesis of Alzheimer's dementia.Expand
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  • Open Access
Doppel-induced cerebellar degeneration in transgenic mice
Doppel (Dpl) is a paralog of the mammalian prion protein (PrP); it is abundant in testes but expressed at low levels in the adult central nervous system. In two Prnp-deficient (Prnp0/0) mouse linesExpand
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  • Open Access