Increased body temperature accelerates aggregation of the (Leu-68–>Gln) mutant cystatin C, the amyloid-forming protein in hereditary cystatin C amyloid angiopathy
Hereditary cystatin C amyloid angiopathy is a dominantly inherited disorder, characterized by dementia, paralysis, and death from cerebral hemorrhage in early adult life. A variant of the cysteine proteinase inhibitor, cystatin C, is deposited as amyloid in the tissues of the patients and their spinal-fluid level of cystatin C is abnormally low. The disease-associated Leu-68-->Gln mutant (L68Q) cy