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Literature summary extracted from

  • Hevel, J.M.; Stewart, J.A.; Gross, K.L.; Ayling, J.E.
    Can the DCoHalpha isozyme compensate in patients with 4a-hydroxy-tetrahydrobiopterin dehydratase/DCoH deficiency? (2006), Mol. Genet. Metab., 88, 38-46.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
4.2.1.96
-
 Homo sapiens

General Stability

EC Number General Stability Organism
4.2.1.96 no decrease in activity upon dilution and storage at 4°C Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
4.2.1.96 0.003
-
 4a-Hydroxy-6(S)-methyltetrahydropterin 10°C, pH 7.4 Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
4.2.1.96 additional information Homo sapiens dehydratase/DCoHalpha, has the kinetic properties necessary for regenerating tetrahydrobiopterin cofactor for phenylalanine hydroxylase. Properties of dehydratase/DCoHalpha are consistent with the hypothesis that the activity of this isozyme could account for the relatively mild symptoms reported for patients with a defect in dehydratase/DCoH ?
-
 ?

Organism

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

Purification (Commentary)

EC Number Purification (Comment) Organism
4.2.1.96 recombinant Homo sapiens

Storage Stability

EC Number Storage Stability Organism
4.2.1.96 4°C, stable Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4.2.1.96 4a-Hydroxy-6(S)-methyltetrahydropterin
-
 Homo sapiens Quinoid 6(S)-methyldihydropterin
-
 ?
4.2.1.96 4alpha-hydroxy-6(S)-methyltetrahydropterin
-
 Homo sapiens ?
-
 ?
4.2.1.96 additional information dehydratase/DCoHalpha, has the kinetic properties necessary for regenerating tetrahydrobiopterin cofactor for phenylalanine hydroxylase. Properties of dehydratase/DCoHalpha are consistent with the hypothesis that the activity of this isozyme could account for the relatively mild symptoms reported for patients with a defect in dehydratase/DCoH Homo sapiens ?
-
 ?

Subunits

EC Number Subunits Comment Organism
4.2.1.96 dimer DCoHalpha Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
4.2.1.96 DCoHalpha
-
 Homo sapiens