AGK siRNA – Acylglycerol Kinase, Mitochondrial (Mouse)

AGK: Lipid kinase that can phosphorylate both monoacylglycerol and diacylglycerol to form lysophosphatidic acid (LPA) and phosphatidic acid (PA), respectively. Does not phosphorylate sphingosine. 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. Defects in AGK are the cause of mitochondrial DNA depletion syndrome type 10 (MTDPS10). 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. Defects in AGK are the cause of cataract, congenital, autosomal recessive type 5 (CATC5). CATC5 consists of an opacification of the crystalline lens of the eye becoming evident at birth. It frequently results in visual impairment or blindness. Opacities vary in morphology, are often confined to a portion of the lens, and may be static or progressive. In general, the more posteriorly located and dense an opacity, the greater the impact on visual function. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 2.7.1.107; Motility/polarity/chemotaxis; Lipid Metabolism - glycerolipid; EC 2.7.1.94; Kinase, lipid

Cellular Component: membrane; intracellular membrane-bound organelle; mitochondrion

Molecular Function: transferase activity; lipid kinase activity; acylglycerol kinase activity; ceramide kinase activity; nucleotide binding; kinase activity; diacylglycerol kinase activity; ATP binding; NAD+ kinase activity

Biological Process: metabolic process; protein kinase C activation; ceramide biosynthetic process; lipid phosphorylation; phosphorylation