NAD kinase is the sole NADP(+) biosynthetic enzyme. A cytosolic form of NAD kinase is already characterised but recently, a mitochondrial form has been found to exist. Mitochondrial NAD kinase 2 (NADK2 aka C5orf33, MNADK, NADKD1) uses ATP to phosphorylate NAD+ to NADP+ (Ohashi et al. 2012). NADK2 is ubiquitously expressed and is more abundant than its cytosolic counterpart. Defects in NADK2 can cause 2,4-dienoyl-CoA reductase deficiency (DECRD), a rare, autosomal recessive, inborn error of polyunsaturated fatty acids and lysine metabolism, resulting in mitochondrial dysfunction (Houten et al. 2014).
Baas, F, Te Brinke, H, Denis, S, Hennekam, RC, Bradley, EJ, Wanders, RJ, Gucsavas-Calikoglu, M, Young, SP, Jongejan, A, Frazier, DM, van Kampen, AH, Houten, SM, Millington, DS
Ohashi, K, Murata, K, Kawai, S
NAD+ kinase activity of NADK2 dimer [mitochondrial matrix]
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