| 简介 | The protein encoded by this gene is a mitochondrial membrane protein involved in lipid and glycerolipid metabolism. The encoded protein is a lipid kinase that catalyzes the formation of phosphatidic and lysophosphatidic acids. Defects in this gene have been associated with mitochondrial DNA depletion syndrome 10. [provided by RefSeq, Feb 2012]
Diseases associated with AGK include Sengers Syndrome and Cataract 38. Among its related pathways are Oncogenic MAPK signaling and Glycerophospholipid biosynthesis. Gene Ontology (GO) annotations related to this gene include NAD+ kinase activity and lipid kinase activity. An important paralog of this gene is CERK.
Lipid kinase that can phosphorylate both monoacylglycerol and diacylglycerol to form lysophosphatidic acid (LPA) and phosphatidic acid (PA), respectively (PubMed:15939762).
Does not phosphorylate sphingosine (PubMed:15939762).
Phosphorylates ceramide (By similarity).
Phosphorylates 1,2-dioleoylglycerol more rapidly than 2,3-dioleoylglycerol (By similarity).
Independently of its lipid kinase activity, acts as a component of the TIM22 complex (PubMed:28712724, 28712726).
In the TIM22 complex, required for the import of a subset of metabolite carriers into mitochondria, such as ANT1/SLC25A4 and SLC25A24, while it is not required for the import of TIMM23 (PubMed:28712724).
Overexpression increases the formation and secretion of LPA, resulting in transactivation of EGFR and activation of the downstream MAPK signaling pathway, leading to increased cell growth (PubMed:15939762).
Overexpressed in prostate cancer, suggesting that it may play a role in initiation and progression of prostate cancer, processes in which lysophosphatidic acid (LPA) plays key roles.
Mitochondrion inner membrane; Peripheral membrane protein {ECO:0000269 PubMed:15939762, 16269826, 28712724, 28712726, 28712724}5
Mitochondrion intermembrane space {ECO:0000269 PubMed:28712724, 28712726}
Note: Localizes in the mitochondrion intermembrane space, where it associates with the inner membrane (PubMed:28712724). It is unclear whether the N-terminal hydrophobic region forms a transmembrane region or associates with the membrane without crossing it (PubMed:28712724, 28712726) {ECO:0000269 PubMed:28712724, 28712726}
Quaternary structure:
Component of the TIM22 complex, which core is composed of TIMM22, associated with TIMM10 (TIMM10A and/or TIMM10B), TIMM9, AGK and TIMM29 (PubMed:28712724, 28712726).
Interacts with SMIM26 (PubMed:37009826). |
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