Universität zu Köln

MacVicar, Thomas and Langer, Thomas ORCID: 0000-0003-1250-1462 (2016). OPA1 processing in cell death and disease - the long and short of it. J. Cell Sci., 129 (12). S. 2297 - 2307. CAMBRIDGE: COMPANY OF BIOLOGISTS LTD. ISSN 1477-9137

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Abstract

The regulation of mitochondrial dynamics by the GTPase OPA1, which is located at the inner mitochondrial membrane, is crucial for adapting mitochondrial function and preserving cellular health. OPA1 governs the delicate balance between fusion and fission in the dynamic mitochondrial network. A disturbance of this balance, often observed under stress and pathologic conditions, causes mitochondrial fragmentation and can ultimately result in cell death. As discussed in this Commentary, these morphological changes are regulated by proteolytic processing of OPA1 by the inner-membrane peptidases YME1L (also known as YME1L1) and OMA1. Long, membrane-bound forms of OPA1 are required for mitochondrial fusion, but their processing to short, soluble forms limits fusion and can facilitate mitochondrial fission. Excessive OPA1 processing by the stress-activated protease OMA1 promotes mitochondrial fragmentation and, if persistent, triggers cell death and tissue degeneration in vivo. The prevention of OMA1-mediated OPA1 processing and mitochondrial fragmentation might thus offer exciting therapeutic potential for human diseases associated with mitochondrial dysfunction.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code MacVicar, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Langer, Thomas UNSPECIFIED orcid.org/0000-0003-1250-1462 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-272235
DOI:	10.1242/jcs.159186
Journal or Publication Title:	J. Cell Sci.
Volume:	129
Number:	12
Page Range:	S. 2297 - 2307
Date:	2016
Publisher:	COMPANY OF BIOLOGISTS LTD
Place of Publication:	CAMBRIDGE
ISSN:	1477-9137
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language MITOCHONDRIAL INNER-MEMBRANE; DOMINANT OPTIC ATROPHY; DYNAMIN-RELATED GTPASE; CYTOCHROME-C RELEASE; M-AAA PROTEASE; PROTEOLYTIC CLEAVAGE; OXIDATIVE-PHOSPHORYLATION; CRISTAE MORPHOGENESIS; DRP1 RECRUITMENT; MAMMALIAN-CELLS Multiple languages Cell Biology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/27223