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Literature summary extracted from

  • Wright, D.J.; Lim, C.K.
    Simultaneous determination of hydroxymethylbilane synthase and uroporphyrinogen III synthase in erythrocytes by high-performance liquid chromatography (1983), Biochem. J., 213, 85-88.
    View publication on PubMedView publication on EuropePMC

Application

EC Number Application Comment Organism
2.5.1.61 medicine a HPLC method for simultaneous determination of porphobilinogen deaminase and uroporphobilinogen III synthase activity. The estimation of porphobilinogen deaminase activity is widely used for the diagnosis of acute intermittent porphyria where the abnormality of the enzyme is the primary genetic defect Homo sapiens

General Stability

EC Number General Stability Organism
4.2.1.75 enzyme reaction linear up to 30 min at 35¦C Homo sapiens

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
4.2.1.75 Mg2+ activates Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
2.5.1.61 porphobilinogen Homo sapiens
-
 ?
-
 ?
4.2.1.75 Hydroxymethylbilane Homo sapiens
-
 ?
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
2.5.1.61 Homo sapiens
-
-
-
4.2.1.75 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
2.5.1.61 erythrocyte
-
 Homo sapiens
-
4.2.1.75 erythrocyte
-
 Homo sapiens
-

Storage Stability

EC Number Storage Stability Organism
4.2.1.75 -20¦C, several months Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2.5.1.61 4 porphobilinogen in the presence of uroporphyrinogen III cosynthase, EC 4.2.1.75 Homo sapiens uroporphyrinogen III + 4 NH3
-
 ?
2.5.1.61 4 porphobilinogen + H2O
-
 Homo sapiens hydroxylmethylbilane + 4 NH3
-
 ?
2.5.1.61 porphobilinogen
-
 Homo sapiens ?
-
 ?
4.2.1.75 Hydroxymethylbilane
-
 Homo sapiens Uroporphyrinogen III
-
 ?
4.2.1.75 Hydroxymethylbilane
-
 Homo sapiens ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
2.5.1.61 HMB-S
-
 Homo sapiens

Temperature Stability [°C]

EC Number Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
4.2.1.75 35
-
 enzyme reaction linear up to 30 min Homo sapiens

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
2.5.1.61 additional information
-
-
 Homo sapiens
4.2.1.75 7.8 7.95
-
 Homo sapiens