Atypical kinase COQ8A, mitochondrial

Definition:

Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. Its substrate specificity is unclear: does not show any protein kinase activity. Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway, as suggested by its ability to bind coenzyme Q lipid intermediates. Shows an unusual selectivity for binding ADP over ATP.

References:

[1]. Brody Wheeler, et al. Preparation and characterization of human ADCK3, a putative atypical kinase. Protein Expr Purif. 2015 Apr;108:13-17. [Content Brief]

[2]. Jonathan A Stefely, et al. Cerebellar Ataxia and Coenzyme Q Deficiency through Loss of Unorthodox Kinase Activity. Mol Cell. 2016 Aug 18;63(4):608-620. [Content Brief]

[3]. Jonathan A Stefely, et al. Mitochondrial ADCK3 employs an atypical protein kinase-like fold to enable coenzyme Q biosynthesis. Mol Cell. 2015 Jan 8;57(1):83-94. [Content Brief]

[4]. Letian X Xie, et al. Expression of the human atypical kinase ADCK3 rescues coenzyme Q biosynthesis and phosphorylation of Coq polypeptides in yeast coq8 mutants. Biochim Biophys Acta. 2011 May;1811(5):348-60. [Content Brief]

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