NADK2 dimer phosphorylates NAD+ to NADP+

Stable Identifier
R-HSA-8955030
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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).

Literature References
PubMed ID Title Journal Year
23212377 Identification and characterization of a human mitochondrial NAD kinase

Ohashi, K, Kawai, S, Murata, K

Nat Commun 2012
24847004 Mitochondrial NADP(H) deficiency due to a mutation in NADK2 causes dienoyl-CoA reductase deficiency with hyperlysinemia

Houten, SM, Denis, S, Te Brinke, H, Jongejan, A, van Kampen, AH, Bradley, EJ, Baas, F, Hennekam, RC, Millington, DS, Young, SP, Frazier, DM, Gucsavas-Calikoglu, M, Wanders, RJ

Hum. Mol. Genet. 2014
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Catalyst Activity
Title
NAD+ kinase activity of NADK2 dimer [mitochondrial matrix]
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Activity
Orthologous Events
Cross References
Rhea
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Created