Biotinidase deficiency: A novel vitamin recycling defect

  • B. Wolf
  • R. E. Grier
  • J. R. Secor McVoy
  • G. S. Heard
Section III: Biotin


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.


Public Health Internal Medicine Metabolic Disease Biotin Biochemical Feature 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© SSIEM and MTP Press Limited 1985

Authors and Affiliations

  • B. Wolf
    • 1
  • R. E. Grier
    • 1
  • J. R. Secor McVoy
    • 1
  • G. S. Heard
    • 1
  1. 1.Departments of Human Genetics and PediatricsMedical College of VirginiaRichmondUSA

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