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- W1994920474 abstract "Abstract Two types of familial cerebral amyloid angiopathy or hereditary cerebral hemorrhage with amyloidosis (HCHWA) have been described: the Icelandic type (HCHWA‐I) and the Dutch type (HCHWA‐D). Both are autosomal‐dominant forms of amyloidosis restricted to the small vasculature of the brain and are clinically characterized by recurrent strokes leading to an early death. In spite of their clinico‐pathological similarities, the amyloid fibrils are structurally different. In the case of HCHWA‐I, the amyloid protein is a degradation product of Cystatin C, a normal serum protein and an inhibitor of cysteine proteases. On the other hand, the amyloid protein in HCHWA‐D has been shown to be related to Alzheimerapos;s disease β‐protein, and preliminary observations indicate that similar amyloid subunits are present in sporadic cerebral amyloid angiopathy and asymptomatic elderly people with amyloidosis. The processing of the β‐protein precursor in different cell types may account for the heterogeneous pattern of clinical manifestations and amyloid deposition in this group of disorders, which we designate “beta‐amayloid diseases.”" @default.
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- W1994920474 date "1988-01-01" @default.
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- W1994920474 title "Human cerebral amyloidosis" @default.
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- W1994920474 doi "https://doi.org/10.1002/ddr.430150205" @default.
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