Biotin--protein ligase

Definition:

Biotin--protein ligase catalyzing the biotinylation of the 4 biotin-dependent carboxylases acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, and methylcrotonyl-CoA carboxylase.

References:

[1]. Y Suzuki, et al. Isolation and characterization of mutations in the human holocarboxylase synthetase cDNA. Nat Genet. 1994 Oct;8(2):122-8. [Content Brief]

[2]. O Sakamoto, et al. Relationship between kinetic properties of mutant enzyme and biochemical and clinical responsiveness to biotin in holocarboxylase synthetase deficiency. Pediatr Res. 1999 Dec;46(6):671-6. [Content Brief]

[3]. A León-Del-Rio, et al. Isolation of a cDNA encoding human holocarboxylase synthetase by functional complementation of a biotin auxotroph of Escherichia coli. Proc Natl Acad Sci U S A. 1995 May 9;92(10):4626-30. [Content Brief]

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