Universität zu Köln

Engqvist, Martin K. M., Esser, Christian, Maier, Alexander, Lercher, Martin J. and Maurino, Veronica G. (2014). Mitochondrial 2-hydroxyglutarate metabolism. Mitochondrion, 19. S. 275 - 282. OXFORD: ELSEVIER SCI LTD. ISSN 1872-8278

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Abstract

2-Hydroxyglutarate (2-HG) is a five-carbon dicarboxylic acid with a hydroxyl group at the alpha position, which forms a stereocenter in this molecule. Although the existence of mitochondrial D- and L-2HG metabolisms has long been known in different eukaryotes, the biosynthetic pathways, especially in plants, have not been completely elucidated. While D-2HG is involved in intermediary metabolism, L-2HG may not have a cellular function but it needs to be recycled through a metabolic repair reaction. Independent of their metabolic origin, D- and L-2HG are oxidized in plant mitochondria to 2-ketoglutarate through the action of two stereospecific enzymes, D- and L-2-hydroxyacid dehydrogenases. While plants are to a large extent unaffected by high cellular concentrations of D-2HG, deficiencies in the metabolism of D- and L-2HG result in fatal disorders in humans. We present current data gathered on plant D- and L-2HG metabolisms and relate it to existing knowledge on 2HG metabolism in other organisms. We focus on the metabolic origin of these compounds, the mitochondrial catabolic steps catalyzed by the stereospecific dehydrogenases, and phylogenetic relationships between different studied 2-hydroxyacid dehydrogenases. (C) 2014 Elsevier B.V. and Mitochondria Research Society. All rights reserved.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Engqvist, Martin K. M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Esser, Christian UNSPECIFIED UNSPECIFIED UNSPECIFIED Maier, Alexander UNSPECIFIED UNSPECIFIED UNSPECIFIED Lercher, Martin J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Maurino, Veronica G. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-423986
DOI:	10.1016/j.mito.2014.02.009
Journal or Publication Title:	Mitochondrion
Volume:	19
Page Range:	S. 275 - 282
Date:	2014
Publisher:	ELSEVIER SCI LTD
Place of Publication:	OXFORD
ISSN:	1872-8278
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language REDUCTASE-SACCHAROPINE DEHYDROGENASE; LYSINE-KETOGLUTARATE REDUCTASE; ELECTRON-TRANSFER FLAVOPROTEIN; D-LACTATE DEHYDROGENASE; ALPHA-HYDROXY ACIDS; L-2-HYDROXYGLUTARIC ACIDURIA; ARABIDOPSIS-THALIANA; D-2-HYDROXYGLUTARIC ACIDURIA; IDH2 MUTATIONS; ORGANIC-ACIDS Multiple languages Cell Biology; Genetics & Heredity Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/42398