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Literature summary for 4.2.1.75 extracted from

  • Ben Bdira, F.; Gonzalez, E.; Pluta, P.; Lain, A.; Sanz-Parra, A.; Falcon-Perez, J.M.; Millet, O.
    Tuning intracellular homeostasis of human uroporphyrinogen III synthase by enzyme engineering at a single hotspot of congenital erythropoietic porphyria (2014), Hum. Mol. Genet., 23, 5805-5813.
    View publication on PubMed
Search for more literature for 4.2.1.75

Cloned(Commentary)

Cloned (Comment) Organism
expressed in Escherichia coli C41(DE3) cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
C73R the substitution drastically reduces the enzyme activity (1500fold) and stability compared to the wild type enzyme Homo sapiens
C73R/A69E the mutant shows reduced activity compared to the wild type enzyme Homo sapiens
C73R/L43D the mutant shows slightly increased activity compared to the wild type enzyme Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.05
-
 Hydroxymethylbilane mutant enzyme C73R/A69E, pH and temperature not specified in the publication Homo sapiens
0.06
-
 Hydroxymethylbilane wild type enzyme, pH and temperature not specified in the publication Homo sapiens
0.07
-
 Hydroxymethylbilane mutant enzyme C73R/L43D, pH and temperature not specified in the publication Homo sapiens
10
-
 Hydroxymethylbilane Km above 10 mM, mutant enzyme C73R, pH and temperature not specified in the publication Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
37000
-
 x * 37000, SDS-PAGE Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
hydroxymethylbilane Homo sapiens
-
 uroporphyrinogen III + H2O
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens P10746
-
-

Purification (Commentary)

Purification (Comment) Organism
Ni-NTA column chromatography and Superdex 75 gel filtration Homo sapiens

Source Tissue

Source Tissue Comment Organism Textmining

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
hydroxymethylbilane
-
 Homo sapiens uroporphyrinogen III + H2O
-
 ?

Subunits

Subunits Comment Organism
? x * 37000, SDS-PAGE Homo sapiens

Synonyms

Synonyms Comment Organism
UROIIIS
-
 Homo sapiens
Uroporphyrinogen III synthase
-
 Homo sapiens

Turnover Number [1/s]

Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
10
-
 Hydroxymethylbilane Km above 10 mM, mutant enzyme C73R, pH and temperature not specified in the publication Homo sapiens
170
-
 Hydroxymethylbilane mutant enzyme C73R/A69E, pH and temperature not specified in the publication Homo sapiens
890
-
 Hydroxymethylbilane wild type enzyme, pH and temperature not specified in the publication Homo sapiens
1200
-
 Hydroxymethylbilane mutant enzyme C73R/L43D, pH and temperature not specified in the publication Homo sapiens

General Information

General Information Comment Organism
malfunction congenital erythropoietic porphyria results from a deficiency in uroporphyrinogen III synthase enzyme activity Homo sapiens

kcat/KM [mM/s]

kcat/KM Value [1/mMs-1] kcat/KM Value Maximum [1/mMs-1] Substrate Comment Organism Structure
1
-
 Hydroxymethylbilane kcat/Km less than 1 1/sec*mM, mutant enzyme C73R, pH and temperature not specified in the publication Homo sapiens
3700
-
 Hydroxymethylbilane mutant enzyme C73R/A69E, pH and temperature not specified in the publication Homo sapiens
15000
-
 Hydroxymethylbilane wild type enzyme, pH and temperature not specified in the publication Homo sapiens
16000
-
 Hydroxymethylbilane mutant enzyme C73R/L43D, pH and temperature not specified in the publication Homo sapiens