Biotinidase: Its role in biotinidase deficiency and biotin metabolism

Barry Wolf*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

39 Scopus citations


Renewed interest in biotinidase, the enzyme responsible for recycling the vitamin biotin, initially came from the discovery of biotinidase deficiency in 1982. Since then, the elucidation of other activities of the enzyme, alternative splicing of the biotinidase gene and differential subcellular localization of the enzyme have prompted speculation and investigations of its other possible functions. The results of these studies have implicated biotinidase in aspects of biotin metabolism, specifically the biotinylation of various proteins, such as histones. Biotinidase may have an important regulatory role(s) in chromatin/DNA function.

Original languageEnglish (US)
Pages (from-to)441-445
Number of pages5
JournalJournal of Nutritional Biochemistry
Issue number7
StatePublished - Jul 2005


  • Alternative splicing
  • Biotin
  • Biotinidase
  • Biotinidase deficiency
  • Biotinylation
  • Subcellular localization

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Molecular Biology
  • Nutrition and Dietetics
  • Clinical Biochemistry


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