EC Number | Application | Comment | Organism |
---|---|---|---|
3.5.1.12 | medicine | plasma biotinidase activity assay for diagnosis and screen newborn infants for biotinidase deficiency, treatment of inborn metabolism errors with large doses of biotin | Homo sapiens |
EC Number | Cloned (Comment) | Organism |
---|---|---|
3.5.1.12 | cDNA from a liver library encoding biotinidase cloned and sequenced | Homo sapiens |
EC Number | Inhibitors | Comment | Organism | Structure |
---|---|---|---|---|
3.5.1.12 | biotin | competitive inhibition at acidic pH, no inhibition at ph 7.4 with p-(N-biotinylamino)benzoic acid as substrate | Homo sapiens | |
3.5.1.12 | biotin | competitive inhibition at acidic pH, no inhibition at ph 7.4 with p-(N-biotinylamino)benzoic acid as substrate | Sus scrofa | |
3.5.1.12 | monoiodoacetate | - |
Homo sapiens | |
3.5.1.12 | monoiodoacetate | - |
Sus scrofa | |
3.5.1.12 | p-hydroxymercuribenzoate | - |
Homo sapiens | |
3.5.1.12 | p-hydroxymercuribenzoate | - |
Rattus rattus | |
3.5.1.12 | p-hydroxymercuribenzoate | - |
Sus scrofa |
EC Number | KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|---|
3.5.1.12 | 0.005 | - |
epsilon-N-biotinyl-L-lysine | - |
Homo sapiens | |
3.5.1.12 | 0.0062 | - |
epsilon-N-biotinyl-L-lysine | human plasma | Homo sapiens | |
3.5.1.12 | 0.0078 | - |
epsilon-N-biotinyl-L-lysine | - |
Homo sapiens | |
3.5.1.12 | 0.01 | - |
biotinyl-4-aminobenzoic acid | - |
Sus scrofa | |
3.5.1.12 | 0.01 | - |
biotinyl-4-aminobenzoic acid | human plasma | Homo sapiens | |
3.5.1.12 | 0.015 | - |
p-(N-biotinylamino)benzoic acid | crude liver extract | Rattus rattus |
EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|---|
3.5.1.12 | microsome | rough endoplasmatic reticulum and Golgi apparatus | Rattus rattus | - |
- |
EC Number | Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|---|
3.5.1.12 | 56770 | - |
estimated from amino acid sequence | Homo sapiens |
3.5.1.12 | 66000 | 76000 | - |
Homo sapiens |
3.5.1.12 | 70000 | 80000 | glycosylated enzyme | Homo sapiens |
3.5.1.12 | 110000 | - |
isoenzyme, co-purified in addition to a 76000 kD biotinidase | Homo sapiens |
EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
3.5.1.12 | biotin amide + H2O | Homo sapiens | - |
? | - |
? | |
3.5.1.12 | biotin amide + H2O | Sus scrofa | - |
? | - |
? | |
3.5.1.12 | biotin amide + H2O | Rattus rattus | - |
? | - |
? | |
3.5.1.12 | epsilon-N-biotinyl-L-lysine + H2O | Homo sapiens | - |
? | - |
? | |
3.5.1.12 | epsilon-N-biotinyl-L-lysine + H2O | Sus scrofa | - |
? | - |
? | |
3.5.1.12 | epsilon-N-biotinyl-L-lysine + H2O | Rattus rattus | - |
? | - |
? | |
3.5.1.12 | Leu-enkephalin + H2O | Homo sapiens | - |
? | - |
? | |
3.5.1.12 | Met-enkephalin + H2O | Homo sapiens | suitable natural substrate for biotinidase | ? | - |
? |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
3.5.1.12 | Homo sapiens | - |
human | - |
3.5.1.12 | Rattus rattus | - |
rat | - |
3.5.1.12 | Sus scrofa | - |
- |
- |
EC Number | Source Tissue | Comment | Organism | Textmining |
---|---|---|---|---|
3.5.1.12 | adrenal gland | - |
Homo sapiens | - |
3.5.1.12 | blood | serum, plasma, leukocytes | Homo sapiens | - |
3.5.1.12 | blood | serum, plasma, leukocytes | Sus scrofa | - |
3.5.1.12 | blood | serum, plasma, leukocytes | Rattus rattus | - |
3.5.1.12 | brain | - |
Homo sapiens | - |
3.5.1.12 | brain | - |
Sus scrofa | - |
3.5.1.12 | brain | - |
Rattus rattus | - |
3.5.1.12 | fibroblast | - |
Homo sapiens | - |
3.5.1.12 | hepatocyte | - |
Homo sapiens | - |
3.5.1.12 | kidney | - |
Homo sapiens | - |
3.5.1.12 | kidney | - |
Sus scrofa | - |
3.5.1.12 | kidney | - |
Rattus rattus | - |
3.5.1.12 | liver | - |
Homo sapiens | - |
3.5.1.12 | liver | - |
Sus scrofa | - |
3.5.1.12 | liver | - |
Rattus rattus | - |
3.5.1.12 | pancreas | - |
Rattus rattus | - |
EC Number | Storage Stability | Organism |
---|---|---|
3.5.1.12 | 4°C, 0.1 M phosphate buffer pH 6.0, 1 mM 2-mercaptoethanol, 1 mM EDTA, 1 month without loss of activity | Homo sapiens |
EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
3.5.1.12 | biotin amide + H2O | - |
Homo sapiens | ? | - |
? | |
3.5.1.12 | biotin amide + H2O | - |
Sus scrofa | ? | - |
? | |
3.5.1.12 | biotin amide + H2O | - |
Rattus rattus | ? | - |
? | |
3.5.1.12 | biotinyl-monoiodotyramine + H2O | radioactive assay for early diagnosis of biotinidase defiency in serum | Homo sapiens | biotin + monoiodotyramine | - |
? | |
3.5.1.12 | epsilon-N-biotinyl-L-lysine + H2O | - |
Homo sapiens | biotin + L-lysine | - |
ir | |
3.5.1.12 | epsilon-N-biotinyl-L-lysine + H2O | - |
Sus scrofa | biotin + L-lysine | - |
ir | |
3.5.1.12 | epsilon-N-biotinyl-L-lysine + H2O | - |
Rattus rattus | biotin + L-lysine | - |
ir | |
3.5.1.12 | epsilon-N-biotinyl-L-lysine + H2O | - |
Homo sapiens | ? | - |
? | |
3.5.1.12 | epsilon-N-biotinyl-L-lysine + H2O | - |
Sus scrofa | ? | - |
? | |
3.5.1.12 | epsilon-N-biotinyl-L-lysine + H2O | - |
Rattus rattus | ? | - |
? | |
3.5.1.12 | Leu-enkephalin + H2O | - |
Homo sapiens | ? | - |
? | |
3.5.1.12 | Met-enkephalin + H2O | suitable natural substrate for biotinidase | Homo sapiens | ? | - |
? | |
3.5.1.12 | p-(N-biotinylamino)benzoic acid + H2O | - |
Homo sapiens | biotin + p-aminobenzoic acid | - |
ir | |
3.5.1.12 | p-(N-biotinylamino)benzoic acid + H2O | - |
Sus scrofa | biotin + p-aminobenzoic acid | - |
ir | |
3.5.1.12 | p-(N-biotinylamino)benzoic acid + H2O | - |
Rattus rattus | biotin + p-aminobenzoic acid | - |
? |
EC Number | Temperature Stability Minimum [°C] | Temperature Stability Maximum [°C] | Comment | Organism |
---|---|---|---|---|
3.5.1.12 | 60 | 70 | - |
Rattus rattus |
3.5.1.12 | 60 | 70 | stable up to 30 min | Homo sapiens |
3.5.1.12 | 60 | 70 | stable up to 30 min | Sus scrofa |
3.5.1.12 | 60 | 70 | 15 min 60°C 60% activity lost, 70°C activity completely lost | Homo sapiens |
EC Number | pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|---|
3.5.1.12 | 4.5 | 6 | with natural substrate biocytin | Homo sapiens |
3.5.1.12 | 5 | 8 | p-(N-biotinylamino)benzoic acid as substrate | Homo sapiens |