G.P.13.15 In sporadic inclusion-body myositis muscle-fiber (s-IBM) cytoplasm, cytochrome C aggregates with α-synuclein and amyloid-β precursor protein (AβPP), but does not activate caspase-3

Abstract

Cytoplasmic aggregates containing ubiquitinated, misfolded proteins, including AβPP, amyloid-β (Aβ) and α-synuclein (α-syn) are characteristic of s-IBM muscle fibers. Mitochondrial abnormalities are also present. Cytochrome C (Cyt C) abnormalities had not been studied. Cyt C regulates electron transport in mitochondria. Its release from mitochondria is essential to activate apoptotic cell death in mononucleated cells. In Lewy Bodies (LBs) of parkinson brain, Cyt C associates with α-syn, and was proposed to facilitate α-syn aggregation.