Entrez Summary: The protein encoded by this gene functions to recycle protein-bound biotin by cleaving biocytin (biotin-epsilon-lysine), a normal product of carboxylase degradation, resulting in regeneration of free biotin. The encoded protein has also been shown to have biotinyl transferase activity. Mutations in this gene are associated with biotinidase deficiency. Multiple transcript variants encoding different isoforms have been described. [provided by RefSeq, Aug 2013].
UniProt Summary: Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.
Pfam DomainsGO Terms
Pfam Domains
CN hydrolase
GO Terms
biotinidase activity
biotin metabolic process
water-soluble vitamin metabolic process
vitamin metabolic process
coenzyme metabolic process
sulfur compound metabolic process
mitochondrial matrix
cofactor metabolic process
drug metabolic process
monocarboxylic acid metabolic process
cellular amide metabolic process
carboxylic acid metabolic process
central nervous system development
oxoacid metabolic process
organic acid metabolic process
extracellular space
small molecule metabolic process
extracellular region
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)
Compound Hit
No hits were found.
Most Correlated Genes in Chemogenomics
No correlation found to any other genes in chemogenomics.
Tissues where Essential in the Avana Dataset (DepMap 20Q1)
Global Fraction of Cell Lines Where Essential: 0/739