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Literature summary for 3.5.1.12 extracted from
- A sensitive fluorimetric rate assay for biotinidase using a new derivative of biotin, biotynyl-6-aminoquinoline (1984), Anal. Biochem., 140, 69-73.
Application
| Application | Comment | Organism |
|---|---|---|
| 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 |
Inhibitors
| Inhibitors | Comment | Organism | Structure |
|---|---|---|---|
| biotin | competitive inhibition at acidic pH, no inhibition at ph 7.4 with p-(N-biotinylamino)benzoic acid as substrate | Homo sapiens |  |
| biotin | competitive inhibition at acidic pH, no inhibition at ph 7.4 with p-(N-biotinylamino)benzoic acid as substrate | Sus scrofa | |
KM Value [mM]
| KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|
| 0.008 | - |
epsilon-N-biotinyl-L-lysine | - |
Sus scrofa | |
| 0.01 | - |
epsilon-N-biotinyl-L-lysine | porcine liver | Sus scrofa | |
| 0.01 | - |
biotinyl-4-aminobenzoic acid | porcine liver, human serum | Homo sapiens | |
| 0.01 | - |
biotinyl-4-aminobenzoic acid | porcine liver, human serum | Sus scrofa | |
| 0.016 | - |
epsilon-N-biotinyl-L-lysine | - |
Sus scrofa | |
| 0.022 | - |
Biotinyl-6-aminoquinoline | human plasma | Homo sapiens | |
| 0.055 | - |
biotinyl-4-aminobenzoic acid | porcine serum | Sus scrofa | |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| biotin amide + H2O | Homo sapiens | - |
? | - |
? | |
| biotin amide + H2O | Sus scrofa | - |
? | - |
? | |
| epsilon-N-biotinyl-L-lysine + H2O | Homo sapiens | - |
? | - |
? | |
| epsilon-N-biotinyl-L-lysine + H2O | Sus scrofa | - |
? | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
human | - |
| Sus scrofa | - |
pig | - |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| blood | serum, plasma, leukocytes | Homo sapiens | - |
| blood | serum, plasma, leukocytes | Sus scrofa | - |
| fibroblast | - |
Homo sapiens | - |
| kidney | - |
Homo sapiens | - |
| kidney | - |
Sus scrofa | - |
| liver | - |
Homo sapiens | - |
| liver | - |
Sus scrofa | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| biotin amide + H2O | - |
Homo sapiens | ? | - |
? | |
| biotin amide + H2O | - |
Sus scrofa | ? | - |
? | |
| biotinyl-4-aminobenzoic acid + H2O | - |
Sus scrofa | biotin + 4-aminobenzoic acid | - |
? | |
| biotinyl-4-aminobenzoic acid + H2O | colorimetric synthetic substrate | Homo sapiens | biotin + 4-aminobenzoic acid | - |
? | |
| biotinyl-6-aminoquinoline + H2O | BAQ, fluorogenic substrate as assay for biotinidase | Homo sapiens | biotin + 6-aminoquinoline | - |
? | |
| epsilon-N-biotinyl-L-lysine + H2O | - |
Homo sapiens | biotin + L-lysine | - |
ir | |
| epsilon-N-biotinyl-L-lysine + H2O | - |
Sus scrofa | biotin + L-lysine | - |
ir | |
| epsilon-N-biotinyl-L-lysine + H2O | - |
Homo sapiens | ? | - |
? | |
| epsilon-N-biotinyl-L-lysine + H2O | - |
Sus scrofa | ? | - |
? | |
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