Biotinidase deficiency: A novel vitamin recycling defect

  title={Biotinidase deficiency: A novel vitamin recycling defect},
  author={B. Wolf and R. E. Grier and J. S. Secor McVoy and G. S. Heard},
  journal={Journal of Inherited Metabolic Disease},
  • B. Wolf, R. E. Grier, +1 author G. S. Heard
  • Published 2005
  • Biology, Medicine
  • Journal of Inherited Metabolic Disease
  • The recent finding that biotinidase deficiency is the primary biochemical defect in late-onset multiple carboxylase deficiency has stimulated new interest in the inherited disorders of biotin-dependent carboxylases. The clinical and biochemical features of biotinidase deficiency are discussed. We also speculate about two exciting areas currently being investigated: the localization of action of biotinidase, and the possible role of the enzyme as a binding or carrier protein for biotin. 
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