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Literature summary extracted from

  • Hymes, J.; Fleischhauer, K.; Wolf, B.
    Biotinidase in serum and tissues (1997), Methods Enzymol., 279, 422-435.
    View publication on PubMed

Application

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

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.5.1.12 cDNA from a liver library encoding biotinidase cloned and sequenced Homo sapiens

Inhibitors

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

KM Value [mM]

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

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.5.1.12 microsome rough endoplasmatic reticulum and Golgi apparatus Rattus rattus
-
-

Molecular Weight [Da]

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

Natural Substrates/ Products (Substrates)

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 ?
-
?

Organism

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
-
-
-

Source Tissue

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
-

Storage Stability

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

Substrates and Products (Substrate)

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
-
?

Temperature Stability [°C]

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

pH Optimum

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