A Novel Amyloidogenic Variant of Apolipoprotein AI : Implications for a Conformational Change Leading to Cardiomyopathy

摘要: Amyloidosis is a broad term for group of diseases which have in common the extracellular deposition pathological insoluble fibrillar proteins organs and tissues. 1 Although unrelated primary sequence metabolic roles, almost all amyloid exhibit β-pleated sheet secondary structure their normal environments green birefringence on polarization microscopy after Congo red staining, deposit as nonbranching, insoluble, highly ordered fibrils. 2,3 The protein fibril pathogenesis disease unique each type amyloid. mechanism(s) by such diverse array with different roles become fibrils, damaging tissues ultimately causing death, are unknown.