Biotinidase: its role in biotinidase deficiency and biotin metabolism✩

Abstract 

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.

Keywords: Biotinidase, Biotinidase deficiency, Biotin, Alternative splicing, Subcellular localization, Biotinylation