Application | Comment | Organism |
---|---|---|
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 | Comment | Organism |
---|---|---|
additional information | naturally occuring missense mutations resulting in profound biotinidase deficiency | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
biocytin + H2O | Homo sapiens | natural substrate, biotinidase is responsible for cleaving biocytin, thereby liberating and recycling biotin | biotin + L-lysine | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Posttranslational Modification | Comment | Organism |
---|---|---|
glycoprotein | - |
Homo sapiens |
Purification (Comment) | Organism |
---|---|
- |
Homo sapiens |
Reaction | Comment | Organism | Reaction ID |
---|---|---|---|
biotin amide + H2O = biotin + NH3 | also acts on biotin esters | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
biocytin + H2O | natural substrate | Homo sapiens | biotin + L-lysine | - |
? | |
biocytin + H2O | natural substrate, biotinidase is responsible for cleaving biocytin, thereby liberating and recycling biotin | Homo sapiens | biotin + L-lysine | - |
? | |
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 | - |
? | |
additional information | no hydrolysis of acetamide or p-nitroacetanilide | Homo sapiens | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
monomer | - |
Homo sapiens |