Mitochondrial BTD hydrolyses BCTN
BCTN → Btn + L-Lys
- Reactome
- R-HSA-4167509
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:77172
Inputs
- BCTNCHEBI:195545, C16H28N4O4S, mitochondrial matrix
Outputs
- BtnCHEBI:15956, C10H16N2O3S, mitochondrial matrix
- L-LysCHEBI:32551, C6H15N2O2, mitochondrial matrix
Catalysed by
- biotinidase activity of BTD [mitochondrial matrix]EC 3.5.1.12; UniProt P43251
Literature
- Human serum biotinidase. cDNA cloning, sequence, and characterization J. Biol. Chem., 1994
- Biotinidase deficiency: the enzymatic defect in late-onset multiple carboxylase deficiency Clin. Chim. Acta, 1983
- Evolutionary conservation of biotinidase: implications for the enzyme's structure and subcellular localization Mol. Genet. Metab., 2005