Janos Zempleni, Subhashinee S.K. Wijeratne, Yousef I. Hassan

Research output: Contribution to journalReview articlepeer-review

275 Scopus citations


Biotin is a water-soluble vitamin and serves as a coenzyme for five carboxylases in humans. Biotin is also covalently attached to distinct lysine residues in histones, affecting chromatin structure and mediating gene regulation. This review describes mammalian biotin metabolism, biotin analysis, markers of biotin status, and biological functions of biotin. Proteins such as holocarboxylase synthetase, biotinidase, and the biotin transporters SMVT and MCT1 play crucial roles in biotin homeostasis, and these roles are reviewed here. Possible effects of inadequate biotin intake, drug interactions, and inborn errors of metabolism are discussed, including putative effects on birth defects.

Original languageEnglish (US)
Pages (from-to)36-46
Number of pages11
Issue number1
StatePublished - Jan 2009


  • Biotin
  • Biotinidase
  • Deficiency
  • Holocarboxylase synthetase
  • Metabolism
  • Transport

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Medicine
  • Clinical Biochemistry


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