| Summary: | Familial amyloid angiopathy, or hereditary cerebral hemorrhage with amyloidosis in patients from Iceland (HCHWA-I)', is an autosomal dominant form of amyloidosis that leads to hemorrhagic and thrombotic strokes causing death before the age of 40 yr (1-3). 128 affected members in eight families originating from the same geographic area in Iceland have been identified. The histopathology of HCHWA-I brains demonstrates generalized amyloid angiopathy, similar to cases with HCHWA of Dutch origin (4) and cases of Alzheimer disease, but they are unassociated with neuritic plaques or tangles (2, 5). The amyloid protein extracted from leptomeninges vessel wall of patients with HCHWA-I was shown to be a variant of cystatin C (6). Very recently, amyloid deposits were also found in tissues outside the central nervous system (7). Human cystatin C (8) (previous nomenclature: , y-CSF [9], post--y protein [10], T-trace [11], and post-gamma globulin [12]) is a low-molecular mass protein shown to be a potent inhibitor of lysosomal cysteine proteinases (8, 13). It is present in many tissues and all body fluids (13-15). Cystatin C is secreted into tissue culture
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