Article
Chromosome 13 dementia syndromes as models of neurodegeneration.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis - 1 Dec 2001
Ghiso J, Révész T, Holton J, Rostagno A, Lashley T, Houlden H, Gibb G, Anderton B, Bek T, Bojsen-Møller M, Wood N, Vidal R, Braendgaard H, Plant G, Frangione B
Abstract excerpt
Two hereditary conditions, familial British dementia (FBD) and familial Danish dementia (FDD), are associated with amyloid deposition in the central nervous system and neurodegeneration. The two amyloid proteins, ABri and ADan, are degradation products of the same precursor molecule BriPP bearing different genetic defects, namely a Stop-to-Arg mutation in FBD and a ten-nucleotide duplication-insertion immediately...
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