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Vidal R, Barbeito AG, Miravalle L, Ghetti B. Cerebral amyloid angiopathy and parenchymal amyloid deposition in transgenic mice expressing the Danish mutant form of human BRI2. Brain Pathol. 2009 Jan;19(1):58-68. PubMed.
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Mount Sinai School of Medicine, NYU
This paper shows the generation of a novel model of cerebral (non-Aβ) amyloid deposition. The authors generated transgenic mice expressing a mutant form of the BRI gene, found in patients affected by familial Danish dementia (FDD). FDD is a rare inherited disease that causes progressive dementia that, like AD, is neuropathologically characterized by amyloid deposition (ADan), neurofibrillary tangle formation (identical to that seen in AD), and neuronal cell loss. This model provides an exciting new tool in which to study the abnormal changes in the brain that lead to dementia. Comparing the similarities and differences of these two related neurological diseases may provide important clues to how AD develops.
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