AGK:Mg2+ phosphorylates MAG, DAG

Stable Identifier
Reaction [transition]
Homo sapiens
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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
22284826 Lack of the mitochondrial protein acylglycerol kinase causes Sengers syndrome

Mayr, JA, Haack, TB, Graf, E, Zimmermann, FA, Wieland, T, Haberberger, B, Superti-Furga, A, Kirschner, J, Steinmann, B, Baumgartner, MR, Moroni, I, Lamantea, E, Zeviani, M, Rodenburg, RJ, Smeitink, J, Strom, TM, Meitinger, T, Sperl, W, Prokisch, H

Am. J. Hum. Genet. 2012
25002142 Proteomic mapping of the human mitochondrial intermembrane space in live cells via ratiometric APEX tagging

Hung, V, Zou, P, Rhee, HW, Udeshi, ND, Cracan, V, Svinkina, T, Carr, SA, Mootha, VK, Ting, AY

Mol. Cell 2014
15939762 A novel acylglycerol kinase that produces lysophosphatidic acid modulates cross talk with EGFR in prostate cancer cells

Bektas, M, Payne, SG, Liu, H, Goparaju, S, Milstien, S, Spiegel, S

J. Cell Biol. 2005
1168700 Congenital cataract and mitochondrial myopathy of skeletal and heart muscle associated with lactic acidosis after exercise

Sengers, RC, Trijbels, JM, Willems, JL, Daniels, O, Stadhouders, AM

J. Pediatr. 1975
Participant Of
Catalyst Activity
Catalyst Activity
acylglycerol kinase activity of AGK:Mg2+ [mitochondrial outer membrane]
Physical Entity
Orthologous Events
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