Janos Zempleni, Subhashinee S.K. Wijeratne, Toshinobu Kuroishi

Research output: Chapter in Book/Report/Conference proceedingChapter

26 Scopus citations


Biotin is a water-soluble vitamin and serves as a coenzyme for five carboxylases, which catalyze key steps in the metabolism of fatty acids, glucose, and amino acids in humans. Biotin also regulates gene expression, mediated by biotinylation of lysine residues in histones H2A, H3, and H4, and by various transcription factors. Holocarboxylase synthetase, biotinidase, sodium-dependent multivitamin transporter, and the biotin transporters SMVT and MCT1 play crucial roles in biotin homeostasis in mammals. Human biotin requirements are unknown, and recommendations for dietary intake are based on estimates of biotin intake in apparently healthy populations ("adequate intake"). Individuals carrying mutations in genes coding for holocarboxylase synthetase and biotinidase require lifelong supplementation with pharmacological doses of biotin. Reliable markers for biotin status include the activity of propionyl-CoA carboxylase in lymphocytes, and the urinary excretion of biotin and the metabolite 3-hydroxyisovaleric acid. Anticonvulsants and lipoic acid may interfere with biotin metabolism, thereby increasing biotin requirements. Severe biotin deficiency has been linked to birth defects and impaired immune function in animal studies. Whether these effects of marginal biotin deficiency occur spontaneously in humans remains unclear.

Original languageEnglish (US)
Title of host publicationPresent Knowledge in Nutrition
Subtitle of host publicationTenth Edition
Number of pages16
ISBN (Print)9780470959176
StatePublished - Jun 18 2012


  • Anticonvulsants and lipoic acid, interfering with biotin metabolism
  • Biotin auxotroph microorganisms, biotin analysis, biotin binding to proteins
  • Biotin catabolism pathways
  • Biotin supplementation, gene expression alterations in healthy adults
  • Biotin, as a covalently bound coenzyme for human carboxylases
  • Biotin, gene expression regulation, biotinylation of lysine residues
  • Biotin, water-soluble vitamin, as a coenzyme for five carboxylases
  • Frank biotin deficiency, periorificial dermatitis, conjunctivitis
  • H4K12bio, biotin transporter expression regulation and repression of repeats
  • Serum concentrations and urinary excretions of biotin, on biotin status

ASJC Scopus subject areas

  • Medicine(all)


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