Editing Mitochondrion (section)

=== Mitochondrial fatty acid synthesis ===
{{Main|Mitochondrial fatty acid synthesis}}

Mitochondrial fatty acid synthesis (mtFASII) is essential for cellular respiration and mitochondrial biogenesis.<ref>{{cite journal | vauthors = Kastaniotis AJ, Autio KJ, Kerätär JM, Monteuuis G, Mäkelä AM, Nair RR, Pietikäinen LP, Shvetsova A, Chen Z, Hiltunen JK | title = Mitochondrial fatty acid synthesis, fatty acids and mitochondrial physiology | journal = Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids | volume = 1862 | issue = 1 | pages = 39–48 | date = January 2017 | pmid = 27553474 | doi = 10.1016/j.bbalip.2016.08.011 }}</ref> It is also thought to play a role as a mediator in [[Cell signaling|intracellular signaling]] due to its influence on the levels of bioactive lipids, such as [[Lysophospholipase|lysophospholipids]] and [[sphingolipid]]s.<ref>{{cite journal | vauthors = Clay HB, Parl AK, Mitchell SL, Singh L, Bell LN, Murdock DG | title = Altering the Mitochondrial Fatty Acid Synthesis (mtFASII) Pathway Modulates Cellular Metabolic States and Bioactive Lipid Profiles as Revealed by Metabolomic Profiling | journal = PLOS ONE | volume = 11 | issue = 3 | pages = e0151171 | date = March 2016 | pmid = 26963735 | pmc = 4786287 | doi = 10.1371/journal.pone.0151171 | veditors = Peterson J | doi-access = free | bibcode = 2016PLoSO..1151171C }}</ref>

[[Octanoyl-(acyl-carrier protein):protein N-octanoyltransferase|Octanoyl-ACP]] (C8) is considered to be the most important end product of mtFASII, which also forms the starting substrate of [[lipoic acid]] biosynthesis.<ref name="Nowinski-2018">{{cite journal | vauthors = Nowinski SM, Van Vranken JG, Dove KK, Rutter J | title = Impact of Mitochondrial Fatty Acid Synthesis on Mitochondrial Biogenesis | journal = Current Biology | volume = 28 | issue = 20 | pages = R1212–R1219 | date = October 2018 | pmid = 30352195 | pmc = 6258005 | doi = 10.1016/j.cub.2018.08.022 | bibcode = 2018CBio...28R1212N }}</ref> Since lipoic acid is the cofactor of important mitochondrial enzyme complexes, such as the [[pyruvate dehydrogenase complex]] (PDC), [[Oxoglutarate dehydrogenase complex|α-ketoglutarate dehydrogenase complex]] (OGDC), [[Branched-chain alpha-keto acid dehydrogenase complex|branched-chain α-ketoacid dehydrogenase complex]] (BCKDC), and in the [[glycine cleavage system]] (GCS), mtFASII has an influence on energy metabolism.<ref>{{cite journal | vauthors = Wehbe Z, Behringer S, Alatibi K, Watkins D, Rosenblatt D, Spiekerkoetter U, Tucci S | title = The emerging role of the mitochondrial fatty-acid synthase (mtFASII) in the regulation of energy metabolism | journal = Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids | volume = 1864 | issue = 11 | pages = 1629–1643 | date = November 2019 | pmid = 31376476 | doi = 10.1016/j.bbalip.2019.07.012 }}</ref>

Other products of mtFASII play a role in the regulation of mitochondrial translation, [[Iron-sulfur cluster biosynthesis|FeS cluster biogenesis]] and assembly of oxidative phosphorylation complexes.<ref name="Nowinski-2018" />

Furthermore, with the help of mtFASII and acylated ACP, acetyl-CoA regulates its consumption in mitochondria.<ref name="Nowinski-2018" />