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Literature summary extracted from

  • Swango, K.L.; Hymes, J.; Brown, P.; Wolf, B.
    Amino acid homologies between human biotinidase and bacterial aliphatic amidases: putative identification of the active site of biotinidase (2000), Mol. Genet. Metab., 69, 111-115.
    View publication on PubMed

Application

EC Number Application Comment Organism
3.5.1.12 medicine naturally occuring missense mutations result in biotinidase deficiency, an autosomal recessively inherited disorder in which affected individuals have multiple carboxylase deficiency due to secondary deficiency of free biotin Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.5.1.12 additional information naturally occuring missense mutations resulting in profound biotinidase deficiency Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3.5.1.12 biocytin + H2O Homo sapiens natural substrate, biotinidase is responsible for cleaving biocytin, thereby liberating and recycling biotin biotin + L-lysine
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
3.5.1.12 Homo sapiens
-
-
-

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
3.5.1.12 glycoprotein
-
 Homo sapiens

Purification (Commentary)

EC Number Purification (Comment) Organism
3.5.1.12
-
 Homo sapiens

Reaction

EC Number Reaction Comment Organism Reaction ID
3.5.1.12 biotin amide + H2O = biotin + NH3 also acts on biotin esters Homo sapiens
a

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.5.1.12 biocytin + H2O natural substrate Homo sapiens biotin + L-lysine
-
 ?
3.5.1.12 biocytin + H2O natural substrate, biotinidase is responsible for cleaving biocytin, thereby liberating and recycling biotin Homo sapiens biotin + L-lysine
-
 ?
3.5.1.12 biotin amide + H2O good substrate, Cys-245 is likely the active site cysteine, formation of a thioester intermediate between biotin and cysteine Homo sapiens biotin + NH3
-
 ?
3.5.1.12 additional information no hydrolysis of acetamide or p-nitroacetanilide Homo sapiens ?
-
 ?

Subunits

EC Number Subunits Comment Organism
3.5.1.12 monomer
-
 Homo sapiens