Universität zu Köln

Richter-Dennerlein, Ricarda, Korwitz, Anne, Haag, Mathias, Tatsuta, Takashi, Dargazanli, Sascha, Baker, Michael, Decker, Thorsten, Lamkemeyer, Tobias, Rugarli, Elena I. ORCID: 0000-0002-5782-1067 and Langer, Thomas ORCID: 0000-0003-1250-1462 (2014). DNAJC19, a Mitochondrial Cochaperone Associated with Cardiomyopathy, Forms a Complex with Prohibitins to Regulate Cardiolipin Remodeling. Cell Metab., 20 (1). S. 158 - 172. CAMBRIDGE: CELL PRESS. ISSN 1932-7420

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Abstract

Prohibitins form large protein and lipid scaffolds in the inner membrane of mitochondria that are required for mitochondrial morphogenesis, neuronal survival, and normal lifespan. Here, we have defined the interactome of PHB2 in mitochondria and identified DNAJC19, mutated in dilated cardiomyopathy with ataxia, as binding partner of PHB complexes. We observed impaired cell growth, defective cristae morphogenesis, and similar transcriptional responses in the absence of either DNAJC19 or PHB2. The loss of PHB/DNAJC19 complexes affects cardiolipin acylation and leads to the accumulation of cardiolipin species with altered acyl chains. Similar defects occur in cells lacking the transacylase tafazzin, which is mutated in Barth syndrome. Our experiments suggest that PHB/DNAJC19 membrane domains regulate cardiolipin remodeling by tafazzin and explain similar clinical symptoms in two inherited cardiomyopathies by an impaired cardiolipin metabolism in mitochondrial membranes.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Richter-Dennerlein, Ricarda UNSPECIFIED UNSPECIFIED UNSPECIFIED Korwitz, Anne UNSPECIFIED UNSPECIFIED UNSPECIFIED Haag, Mathias UNSPECIFIED UNSPECIFIED UNSPECIFIED Tatsuta, Takashi UNSPECIFIED UNSPECIFIED UNSPECIFIED Dargazanli, Sascha UNSPECIFIED UNSPECIFIED UNSPECIFIED Baker, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED Decker, Thorsten UNSPECIFIED UNSPECIFIED UNSPECIFIED Lamkemeyer, Tobias UNSPECIFIED UNSPECIFIED UNSPECIFIED Rugarli, Elena I. UNSPECIFIED orcid.org/0000-0002-5782-1067 UNSPECIFIED Langer, Thomas UNSPECIFIED orcid.org/0000-0003-1250-1462 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-435211
DOI:	10.1016/j.cmet.2014.04.016
Journal or Publication Title:	Cell Metab.
Volume:	20
Number:	1
Page Range:	S. 158 - 172
Date:	2014
Publisher:	CELL PRESS
Place of Publication:	CAMBRIDGE
ISSN:	1932-7420
Language:	English
Faculty:	Faculty of Mathematics and Natural Sciences
Divisions:	Faculty of Mathematics and Natural Sciences > Department of Biology > Institute for Genetics
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language M-AAA PROTEASE; INNER MEMBRANE; BARTH-SYNDROME; PRESEQUENCE TRANSLOCASE; RESPIRATORY-CHAIN; IMPORT MOTOR; FATTY-ACIDS; BIOGENESIS; STRESS; YEAST Multiple languages Cell Biology; Endocrinology & Metabolism Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/43521