Cerebral amyloidosis: amyloid subunits, mutants and phenotypes |
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Authors: | A Rostagno J L Holton T Lashley T Revesz Jorge Ghiso |
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Institution: | (1) Department of Pathology, New York University School of Medicine, New York, NY 10016, USA;(2) Department of Psychiatry, New York University School of Medicine, New York, NY 10016, USA;(3) Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square Brain Bank for Neurological Disorders, University College London, Queen Square, London, WC1N 3BG, UK |
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Abstract: | Cerebral amyloid diseases are part of a complex group of chronic and progressive entities bracketed together under the common
denomination of protein folding disorders and characterized by the intra- and extracellular accumulation of fibrillar aggregates.
Of the more than 25 unrelated proteins known to produce amyloidosis in humans only about a third of them are associated with
cerebral deposits translating in cognitive deficits, dementia, stroke, cerebellar and extrapyramidal signs, or a combination
thereof. The familial forms reviewed herein, although infrequent, provide unique paradigms to examine the role of amyloid
in the mechanism of disease pathogenesis and to dissect the link between vascular and parenchymal amyloid deposition and their
differential contribution to neurodegeneration. |
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Keywords: | |
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