Phosphopantothenate--cysteine ligase

Definition:

Catalyzes the second step in the biosynthesis of coenzyme A from vitamin B5, where cysteine is conjugated to 4'-phosphopantothenate to form 4-phosphopantothenoylcysteine. Has a preference for ATP over CTP as a cosubstrate.

References:

[1]. Narayanan Manoj, et al. Structure of human phosphopantothenoylcysteine synthetase at 2.3 A resolution. Structure. 2003 Aug;11(8):927-36. [Content Brief]

[2]. Matthew Daugherty, et al. Complete reconstitution of the human coenzyme A biosynthetic pathway via comparative genomics. J Biol Chem. 2002 Jun 14;277(24):21431-9. [Content Brief]

[3]. Arcangela Iuso, et al. Mutations in PPCS, Encoding Phosphopantothenoylcysteine Synthetase, Cause Autosomal-Recessive Dilated Cardiomyopathy. Am J Hum Genet. 2018 Jun 7;102(6):1018-1030. [Content Brief]

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