title: Mitochondrial fatty acid synthesis, fatty acids and mitochondrial physiology.
abstract: Mitochondria and fatty acids are tightly connected to a multiplicity of cellular processes that go far beyond mitochondrial fatty acid metabolism. In line with this view, there is hardly any common metabolic disorder that is not associated with disturbed mitochondrial lipid handling. Among other aspects of mitochondrial lipid metabolism, apparently all eukaryotes are capable of carrying out de novo fatty acid synthesis (FAS) in this cellular compartment in an acyl carrier protein (ACP)-dependent manner. The dual localization of FAS in eukaryotic cells raises the questions why eukaryotes have maintained the FAS in mitochondria in addition to the "classic" cytoplasmic FAS and what the products are that cannot be substituted by delivery of fatty acids of extramitochondrial origin. The current evidence indicates that mitochondrial FAS is essential for cellular respiration and mitochondrial biogenesis. Although both β-oxidation and FAS utilize thioester chemistry, CoA acts as acyl-group carrier in the breakdown pathway whereas ACP assumes this role in the synthetic direction. This arrangement metabolically separates these two pathways running towards opposite directions and prevents futile cycling. A role of this pathway in mitochondrial metabolic sensing has recently been proposed. This article is part of a Special Issue entitled: Lipids of Mitochondria edited by Guenther Daum.
authors.affiliation: Faculty of Biochemistry and Molecular Medicine, Biocenter Oulu, University of Oulu, Oulu, Finland. Electronic address: alexander.kastaniotis@oulu.fi.
authors.forename: Alexander J
authors.initials: AJ
authors.lastname: Kastaniotis
authors.affiliation: Faculty of Biochemistry and Molecular Medicine, Biocenter Oulu, University of Oulu, Oulu, Finland.
authors.forename: Kaija J
authors.initials: KJ
authors.lastname: Autio
authors.affiliation: Faculty of Biochemistry and Molecular Medicine, Biocenter Oulu, University of Oulu, Oulu, Finland.
authors.forename: Juha M
authors.initials: JM
authors.lastname: Kerätär
authors.affiliation: Faculty of Biochemistry and Molecular Medicine, Biocenter Oulu, University of Oulu, Oulu, Finland.
authors.forename: Geoffray
authors.initials: G
authors.lastname: Monteuuis
authors.affiliation: Faculty of Biochemistry and Molecular Medicine, Biocenter Oulu, University of Oulu, Oulu, Finland.
authors.forename: Anne M
authors.initials: AM
authors.lastname: Mäkelä
authors.affiliation: Faculty of Biochemistry and Molecular Medicine, Biocenter Oulu, University of Oulu, Oulu, Finland.
authors.forename: Remya R
authors.initials: RR
authors.lastname: Nair
authors.affiliation: Faculty of Biochemistry and Molecular Medicine, Biocenter Oulu, University of Oulu, Oulu, Finland.
authors.forename: Laura P
authors.initials: LP
authors.lastname: Pietikäinen
authors.affiliation: Faculty of Biochemistry and Molecular Medicine, Biocenter Oulu, University of Oulu, Oulu, Finland.
authors.forename: Antonina
authors.initials: A
authors.lastname: Shvetsova
authors.affiliation: State Key Laboratory of Supramolecular Structure and Materials and Institute of Theoretical Chemistry, Jilin University, 2699 Qianjin Street, Changchun 130012, PR China.
authors.forename: Zhijun
authors.initials: Z
authors.lastname: Chen
authors.affiliation: Faculty of Biochemistry and Molecular Medicine, Biocenter Oulu, University of Oulu, Oulu, Finland; State Key Laboratory of Supramolecular Structure and Materials and Institute of Theoretical Chemistry, Jilin University, 2699 Qianjin Street, Changchun 130012, PR China. Electronic address: kalervo.hiltunen@oulu.fi.
authors.forename: J Kalervo
authors.initials: JK
authors.lastname: Hiltunen
mesh_terms: D000213:Acyl Carrier Protein / Q000378:metabolism; D000818:Animals; D019069:Cell Respiration / Q000502:physiology; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D050356:Lipid Metabolism / Q000502:physiology; D050155:Lipogenesis / Q000502:physiology; D008928:Mitochondria / Q000378:metabolism* / Q000502:physiology*; D010084:Oxidation-Reduction
chemical_list: D000213:Acyl Carrier Protein; D005227:Fatty Acids
keywords: Fatty acid synthesis; Lipids; Lipoic acid; Metabolic compartmentalization; Metabolic interaction; Pathways; Respiration; Thioesters