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biotinidase <enzyme> Deficiency may lead to late onset multiple carboxylase deficiency (organic acidemias); exhibits biocytin hydrolase and biotinyl-transferase activities
Registry number: EC 3.5.1.12
Synonym: biocytin hydrolase, biotidinase
(26 Jun 1999)
MeSH(Medical Subject Headings) ¸ÂÃã °Ë»ö (http://www.nlm.nih.gov) °á°ú : 2 ÆäÀÌÁö: 1
  • Biotinidase - »õâ An enzyme which catalyzes the release of BIOTIN from biocytin. In human, defects in the enzyme are the cause of the organic acidemia MULTIPLE CARBOXYLASE DEFICIENCY or BIOTINIDASE DEFICIENCY.
    Synonyms : Biocytin Hydrolase, Hydrolase, Biocytin
  • Biotinidase Deficiency - »õâ The late onset form of MULTIPLE CARBOXYLASE DEFICIENCY (deficiency of the activities of biotin-dependent enzymes propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, and PYRUVATE CARBOXYLASE) due to a defect or deficiency in biotinidase which is essential for recycling BIOTIN.
    Synonyms : Deficiency, Biotinidase, Deficiency, Multiple Carboxylase, Late-Onset, Biotinidase Deficiencies, Deficiencies, Biotinidase, Multiple Carboxylase Deficiency, Late Onset
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biotinidase [EC 3.5.1.12] an enzyme of the hydrolase class essential for the recycling of biotin; it catalyzes the cleavage of biocytin or of biotin in amide linkage with peptide fragments, freeing biotin for reuse. Deficiency of the enzyme, an autosomal recessive trait, results in multiple carboxylase deficiency.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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