Universität zu Köln

Mourier, Arnaud, Motori, Elisa, Brandt, Tobias, Lagouge, Marie, Atanassov, Ilian ORCID: 0000-0001-8259-2545, Galinier, Anne ORCID: 0000-0003-2454-5681, Rappl, Gunter, Brodesser, Susanne, Hultenby, Kjell, Dieterich, Christoph and Larsson, Nils-Goran ORCID: 0000-0001-5100-996X (2015). Mitofusin 2 is required to maintain mitochondrial coenzyme Q levels. J. Cell Biol., 208 (4). S. 429 - 443. NEW YORK: ROCKEFELLER UNIV PRESS. ISSN 1540-8140

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Abstract

Mitochondria form a dynamic network within the cell as a result of balanced fusion and fission. Despite the established role of mitofusins (MFN1 and MFN2) in mitochondrial fusion, only MFN2 has been associated with metabolic and neurodegenerative diseases, which suggests that MFN2 is needed to maintain mitochondrial energy metabolism. The molecular basis for the mitochondrial dysfunction encountered in the absence of MFN2 is not understood. Here we show that loss of MFN2 leads to impaired mitochondrial respiration and reduced ATP production, and that this defective oxidative phosphorylation process unexpectedly originates from a depletion of the mitochondrial coenzyme Q pool. Our study unravels an unexpected and novel role for MFN2 in maintenance of the terpenoid biosynthesis pathway, which is necessary for mitochondrial coenzyme Q biosynthesis. The reduced respiratory chain function in cells lacking MFN2 can be partially rescued by coenzyme Q10 supplementation, which suggests a possible therapeutic strategy for patients with diseases caused by mutations in the Mfn2 gene.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Mourier, Arnaud UNSPECIFIED UNSPECIFIED UNSPECIFIED Motori, Elisa UNSPECIFIED UNSPECIFIED UNSPECIFIED Brandt, Tobias UNSPECIFIED UNSPECIFIED UNSPECIFIED Lagouge, Marie UNSPECIFIED UNSPECIFIED UNSPECIFIED Atanassov, Ilian UNSPECIFIED orcid.org/0000-0001-8259-2545 UNSPECIFIED Galinier, Anne UNSPECIFIED orcid.org/0000-0003-2454-5681 UNSPECIFIED Rappl, Gunter UNSPECIFIED UNSPECIFIED UNSPECIFIED Brodesser, Susanne UNSPECIFIED UNSPECIFIED UNSPECIFIED Hultenby, Kjell UNSPECIFIED UNSPECIFIED UNSPECIFIED Dieterich, Christoph UNSPECIFIED UNSPECIFIED UNSPECIFIED Larsson, Nils-Goran UNSPECIFIED orcid.org/0000-0001-5100-996X UNSPECIFIED
URN:	urn:nbn:de:hbz:38-412383
DOI:	10.1083/jcb.201411100
Journal or Publication Title:	J. Cell Biol.
Volume:	208
Number:	4
Page Range:	S. 429 - 443
Date:	2015
Publisher:	ROCKEFELLER UNIV PRESS
Place of Publication:	NEW YORK
ISSN:	1540-8140
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language ENDOPLASMIC-RETICULUM; COUPLING DEFECT; UBIQUINONE; FUSION; CHAIN; IDENTIFICATION; BIOSYNTHESIS; LOCALIZATION; DEGENERATION; PEROXISOMES Multiple languages Cell Biology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/41238