Reactome | NADK2 dimer phosphorylates NAD+ to NADP+

NADK2 dimer phosphorylates NAD+ to NADP+

Stable Identifier

R-HSA-8955030

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

NAD(+) + ATP => ADP + NADP(+) + H(+)

ReviewStatus

4/5

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NADK2 dimer phosphorylates NAD+ to NADP+

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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
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, RJA	Hum. Mol. Genet.	2014
23212377	Identification and characterization of a human mitochondrial NAD kinase Ohashi, K, Kawai, S, Murata, K	Nat Commun	2012