Article
A newly formed amyloidogenic fragment due to a stop codon mutation causes familial British dementia.
Annals of the New York Academy of Sciences - 1 Apr 2000
Ghiso J, Vidal R, Rostagno A, Mead S, Révész T, Plant G, Frangione B
Abstract excerpt
Familial British dementia (FBD) is an early-onset autosomal dominant disorder characterized by progressive cognitive impairment, spasticity, and cerebellar ataxia. Hippocampal neurofibrillar degeneration and widespread parenchymal and vascular amyloid deposits are the main neuropathological lesions. Amyloid fibrils are composed of a novel 34 amino acid subunit (ABri) with no sequence identity to any known amyloid...
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