Mitochondrial metalloendopeptidase OMA1 (OMA1) is part of the quality control system in the inner membrane of mitochondria. OMA1 controls the cleavage and function of mitochondrial dynamin-like 120 kDa protein (OPA1), a GTPase required for mitochondrial fusion and regulation of apoptosis (Head et al. 2009). OPA1 is suggested to be antiapoptotic and mutations in OPA1 are the most prevalent cause of dominant optic atrophy (DOA), a progressive eye disease which affects retinal ganglion cells in the optic nerve (Yu-Wai-Man et al. 2009).
Head, B, van der Bliek, AM, Griparic, L, Gandre-Babbe, S, Amiri, M
Yu-Wai-Man, P, Griffiths, PG, Hudson, G, Chinnery, PF
metalloendopeptidase activity of OMA1:Zn2+ [mitochondrial inner membrane]
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