F
IPR040154

Biotinidase/VNN family

InterPro entry
Short nameBiotinidase/VNN
Overlapping
homologous
superfamilies
 
family relationships

Description

Biotinidase catalyses 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 characterised by neurological and cutaneous abnormalities, and can be successfully treated with biotin supplementation
[2, 3]
.

Pantetheinase is an enzyme that hydrolyses 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
[1]
.

Both mammalian biotinidase and pantetheinase share a carbon-nitrogen hydrolase domain with significant similarity to the photoprotein from oceanic squid symplectin
[4, 5]
.

References

1.Alternative spliced variants in the pantetheinase family of genes expressed in human neutrophils. Nitto T, Inoue T, Node K. Gene 426, 57-64, (2008). View articlePMID: 18805469

2.Human serum biotinidase. cDNA cloning, sequence, and characterization. Cole H, Reynolds TR, Lockyer JM, Buck GA, Denson T, Spence JE, Hymes J, Wolf B. J. Biol. Chem. 269, 6566-70, (1994). PMID: 7509806

3.Mutations in BTD causing biotinidase deficiency. Hymes J, Stanley CM, Wolf B. Hum. Mutat. 18, 375-81, (2001). View articlePMID: 11668630

4.A novel photoprotein from oceanic squid (Symplectoteuthis oualaniensis) with sequence similarity to mammalian carbon-nitrogen hydrolase domains. Fujii T, Ahn JY, Kuse M, Mori H, Matsuda T, Isobe M. Biochem. Biophys. Res. Commun. 293, 874-9, (2002). View articlePMID: 12054553

5.Symplectin evolved from multiple duplications in bioluminescent squid. Francis WR, Christianson LM, Haddock SHD. PeerJ 5, e3633, (2017). View articlePMID: 28785521

Cross References

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