Reactome | AGK:Mg2+ phosphorylates MAG, DAG

AGK:Mg2+ phosphorylates MAG, DAG

Stable Identifier

R-HSA-5696074

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial outer membrane, mitochondrial intermembrane space

ReviewStatus

5/5

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The bioactive phospholipids lysophosphatidic acid (LPA) and phosphatidic acid (PA) regulate processes related to cancer pathogenesis. Mitochondrial acylglycerol kinase (AGK) can phosphorylate both monoacylglycerol (MAG) and diacylglycerol (DAG) in the mitochondrial intermembrane space to form LPA and PA, respectively thus may play an important role in the pathophysiology of certain cancers (Bektas et al. 2005). AGK is mitochondrial outer membrane-bound (Hung et al. 2014) and requires Mg2+ as cofactor.
Defects in AGK can cause mitochondrial DNA depletion syndrome 10 (MTDPS10 aka Sengers syndrome; MIM:212350), an autosomal recessive mitochondrial disorder characterized by congenital cataracts, hypertrophic cardiomyopathy, skeletal myopathy, exercise intolerance, and lactic acidosis. Mental development is normal, but affected individuals may die early from cardiomyopathy (Sengers et al. 1975, Mayr et al. 2012).

Literature References

PubMed ID	Title	Journal	Year
25002142	Proteomic mapping of the human mitochondrial intermembrane space in live cells via ratiometric APEX tagging Cracan, V, Rhee, HW, Udeshi, ND, Ting, AY, Svinkina, T, Hung, V, Mootha, VK, Zou, P, Carr, SA	Mol. Cell	2014
22284826	Lack of the mitochondrial protein acylglycerol kinase causes Sengers syndrome Kirschner, J, Mayr, JA, Meitinger, T, Superti-Furga, A, Rodenburg, RJ, Wieland, T, Zeviani, M, Zimmermann, FA, Sperl, W, Smeitink, J, Baumgartner, MR, Graf, E, Haack, TB, Lamantea, E, Moroni, I, Haberberger, B, Prokisch, H, Steinmann, B, Strom, TM	Am. J. Hum. Genet.	2012
15939762	A novel acylglycerol kinase that produces lysophosphatidic acid modulates cross talk with EGFR in prostate cancer cells Liu, H, Spiegel, S, Milstien, S, Goparaju, S, Payne, SG, Bektas, M	J. Cell Biol.	2005
1168700	Congenital cataract and mitochondrial myopathy of skeletal and heart muscle associated with lactic acidosis after exercise Willems, JL, Sengers, RC, Stadhouders, AM, Daniels, O, Trijbels, JM	J. Pediatr.	1975