Accession NumberHMDB01458
Common_NameBiotin amide
DescriptionThe enzyme biotinidase (EC-Number 3.5.1.12 ) is involved in the recycling of the vitamin biotin, cleaving D-biotinylamides and esters, in a reaction including biotin amide and water. (PMID 1719240, 171927) Late-onset multiple carboxylase deficiency (MCD) with biotinidase deficiency is caused by mutation in the biotinidase gene. MCD is an autosomal recessive metabolic disorder characterized primarily by cutaneous and neurologic abnormalities. Symptoms result from the patient's inability to reutilize biotin, a necessary nutrient. (OMIM 253260)
Chemical_IUPAC_Name5-[(1R,2S,5S)-7-oxo-3-thia-6,8-diazabicyclo[3.3.0]oct-2-yl]pentanamide
Chemical FormulaC10H17N3O2S
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