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01-03-2010 | Review

Modeling familial British and Danish dementia

Authors: Holly J. Garringer, Jill Murrell, Luciano D’Adamio, Bernardino Ghetti, Ruben Vidal

Published in: Brain Structure and Function | Issue 2-3/2010

Abstract

Familial British dementia (FBD) and familial Danish dementia (FDD) are two autosomal dominant neurodegenerative diseases caused by mutations in the BRI ₂ gene. FBD and FDD are characterized by widespread cerebral amyloid angiopathy (CAA), parenchymal amyloid deposition, and neurofibrillary tangles. Transgenic mice expressing wild-type and mutant forms of the BRI₂ protein, Bri ₂ knock-in mutant mice, and Bri ₂ gene knock-out mice have been developed. Transgenic mice expressing a human FDD-mutated form of the BRI ₂ gene have partially reproduced the neuropathological lesions observed in FDD. These mice develop extensive CAA, parenchymal amyloid deposition, and neuroinflammation in the central nervous system. These animal models allow the study of the molecular mechanism(s) underlying the neuronal dysfunction in these diseases and allow the development of potential therapeutic approaches for these and related neurodegenerative conditions. In this review, a comprehensive account of the advances in the development of animal models for FBD and FDD and of their relevance to the study of Alzheimer disease is presented.

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Title: Modeling familial British and Danish dementia
Authors: Holly J. Garringer
Jill Murrell
Luciano D’Adamio
Bernardino Ghetti
Ruben Vidal
Publication date: 01-03-2010
Publisher: Springer-Verlag
Published in: Brain Structure and Function / Issue 2-3/2010
Print ISSN: 1863-2653
Electronic ISSN: 1863-2661
DOI: https://doi.org/10.1007/s00429-009-0221-9

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Modeling familial British and Danish dementia

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