Biotinidase functions to recycle biotin in the body by cleaving biocytin (biotin-epsilon-lysine), a normal product of carboxylase degradation, resulting in regeneration of free biotin. Biotinidase has also been shown to have biotinyl-transferase activity. Defects in the biotinidase gene cause multiple carboxylase deficiency. [provided by RefSeq, Jul 2008].
Cowan, Blitzer, Wolf: "Technical standards and guidelines for the diagnosis of biotinidase deficiency." in: Genetics in medicine : official journal of the American College of Medical Genetics, Vol. 12, Issue 7, pp. 464-70, (2010) (PubMed).