Arginine-hydroxylase NDUFAF5, mitochondrial
Definition:
Arginine hydroxylase involved in the assembly of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I, MT-ND1) at early stages. Acts by mediating hydroxylation of 'Arg-111' of NDUFS7. May also have methyltransferase activity (Probable).
References:
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[1]. Virginie F Rhein, et al. NDUFAF5 Hydroxylates NDUFS7 at an Early Stage in the Assembly of Human Complex I. J Biol Chem. 2016 Jul 8;291(28):14851-60. [Content Brief]
[2]. Canny Sugiana, et al. Mutation of C20orf7 disrupts complex I assembly and causes lethal neonatal mitochondrial disease. Am J Hum Genet. 2008 Oct;83(4):468-78. [Content Brief]
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