OMA1 hydrolyses OPA1

Stable Identifier
R-HSA-8867344
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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).

Literature References
PubMed ID Title Journal Year
19001017 Inherited mitochondrial optic neuropathies

Yu-Wai-Man, P, Griffiths, PG, Hudson, G, Chinnery, PF

J. Med. Genet. 2009
20038677 Inducible proteolytic inactivation of OPA1 mediated by the OMA1 protease in mammalian cells

Head, B, Griparic, L, Amiri, M, Gandre-Babbe, S, van der Bliek, AM

J. Cell Biol. 2009
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
metalloendopeptidase activity of OMA1 [mitochondrial inner membrane]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created