Reactome | OMA1 hydrolyses OPA1

OMA1 hydrolyses OPA1

Stable Identifier

R-HSA-8867344

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial inner membrane, mitochondrial matrix

ReviewStatus

5/5

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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
20038677	Inducible proteolytic inactivation of OPA1 mediated by the OMA1 protease in mammalian cells Head, B, van der Bliek, AM, Griparic, L, Gandre-Babbe, S, Amiri, M	J. Cell Biol.	2009
19001017	Inherited mitochondrial optic neuropathies Yu-Wai-Man, P, Griffiths, PG, Hudson, G, Chinnery, PF	J. Med. Genet.	2009