William Todd Penberthy, Mahrou Sadri, Janos Zempleni

Research output: Chapter in Book/Report/Conference proceedingChapter

3 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. Holocarboxylase synthetase (HCS), biotinidase, sodium-dependent multivitamin transporter, and the biotin transporters SMVT and MCT1 play crucial roles in biotin homeostasis in mammals. Biotin also regulates gene expression by biotinylation of lysine residues of histones H2A, H3, and H4. Human biotin requirements are unknown, and recommendations for dietary intake are based on estimates of biotin intake in healthy populations (“adequate intake”). Individuals carrying mutations in genes coding for HCS or biotinidase require lifelong supplementation with pharmacological doses of biotin. Reliable markers for biotin status include the activity of propionyl-CoA carboxylase and methylcrotonyl-CoA carboxylase, but urinary metabolite markers are unreliable measures of biotin deficiency. Anticonvulsants and lipoic acid may interfere with biotin metabolism, thereby increasing biotin requirements. Severe biotin deficiency has been linked to birth defects, multiple sclerosis misdiagnosis, and impaired immune function.

Original languageEnglish (US)
Title of host publicationPresent Knowledge in Nutrition
Subtitle of host publicationBasic Nutrition and Metabolism
Number of pages15
ISBN (Electronic)9780323661621
ISBN (Print)9780128198421
StatePublished - Jan 1 2020


  • Biotin
  • Holocarboxylase
  • Multiple carboxylase deficiency
  • Multiple sclerosis

ASJC Scopus subject areas

  • Health Professions(all)
  • Medicine(all)


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