NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2

Definition:

Acts as a molecular chaperone for mitochondrial complex I assembly. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.

References:

[1]. Saskia J G Hoefs, et al. Baculovirus complementation restores a novel NDUFAF2 mutation causing complex I deficiency. Hum Mutat. 2009 Jul;30(7):E728-36. [Content Brief]

[2]. David A Stroud, et al. Accessory subunits are integral for assembly and function of human mitochondrial complex I. Nature. 2016 Oct 6;538(7623):123-126. [Content Brief]

[3]. Isla Ogilvie, et al. A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy. J Clin Invest. 2005 Oct;115(10):2784-92. [Content Brief]

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