Cerebral amyloid angiopathy and parenchymal amyloid deposition in transgenic mice expressing the Danish mutant form of human BRI2.

Brain Pathology
Ruben VidalBernardino Ghetti

Abstract

Familial Danish dementia (FDD) is an autosomal dominant neurodegenerative disease clinically characterized by the presence of cataracts, hearing impairment, cerebellar ataxia and dementia. Neuropathologically, FDD is characterized by the presence of widespread cerebral amyloid angiopathy (CAA), parenchymal amyloid deposition and neurofibrillary tangles. FDD is caused by a 10-nucleotide duplication-insertion in the BRI(2) gene that generates a larger-than-normal precursor protein, of which the Danish amyloid subunit (ADan) comprises the last 34 amino acids. Here, we describe a transgenic mouse model for FDD (Tg-FDD) in which the mouse Prnp (prion protein) promoter drives the expression of the Danish mutant form of human BRI(2). The main neuropathological findings in Tg-FDD mice are the presence of widespread CAA and parenchymal deposition of ADan. In addition, we observe the presence of amyloid-associated gliosis, an inflammatory response and deposition of oligomeric ADan. As the animals aged, they showed abnormal grooming behavior, an arched back, and walked with a wide-based gait and shorter steps. This mouse model may give insights on the pathogenesis of FDD and will prove useful for the development of therapeutics. Moreover,...Continue Reading

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Citations

Sep 26, 2009·Brain Structure & Function·Holly J GarringerRuben Vidal
Oct 21, 2010·Irish Journal of Medical Science·A T Welzel, D M Walsh
Nov 26, 2010·Proceedings of the National Academy of Sciences of the United States of America·Robert TamayevLuciano D'Adamio
Mar 31, 2012·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Ruben VidalBernardino Ghetti
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Methods Mentioned

BETA
transgenic
PCR
enzyme-linked
dot blot
confocal microscopy

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