Biotinidase/VNN family (IPR040154)

Short name: Biotinidase/VNN

Overlapping homologous superfamilies

Family relationships


Biotinidase catalyzes the hydrolysis of biocytin, the product of biotin-dependent carboxylase degradation, to biotin and lysine. Biotinidase deficiency is an inherited metabolic disorder of biotin recycling that is characterized by neurological and cutaneous abnormalities, and can be successfully treated with biotin supplementation [PMID: 7509806, PMID: 11668630].

Pantetheinase is an enzyme that hydrolyzes pantetheine, an intermediate metabolite of coenzyme A, into pantothenic acid (vitamin B5) and cysteamine. The pantetheinase family consists of pantetheinase/vanin-1/VNN1, GPI-80/VNN2 and vanin-3/VNN3. VNN2 and VNN3 are expressed in human leukocytes [PMID: 18805469].

Both mammalian biotinidase and pantetheinase share a carbon-nitrogen hydrolase domain with significant similarity to the photoprotein from oceanic squid symplectin [PMID: 12054553, PMID: 28785521].

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.