mitochondrial outer membrane,
mitochondrial intermembrane space
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).