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A decamer duplication in the 3′ region of the <i>BRI</i> gene originates an amyloid peptide that is associated with dementia in a Danish kindred

18 Apr 2000

Abstract excerpt

Familial Danish dementia (FDD), also known as heredopathia ophthalmo-oto-encephalica, is an autosomal dominant disorder characterized by cataracts, deafness, progressive ataxia, and dementia. Neuropathological findings include severe widespread cerebral amyloid angiopathy, hippocampal plaques, and neurofibrillary tangles, similar to Alzheimer's disease. N-terminal sequence analysis of isolated leptomeningeal...

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PMID: 10781099
DOI: 10.1073/pnas.080076097

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