DB code: S00237
| CATH domain | 3.20.20.210 : TIM Barrel | Catalytic domain |
|---|---|---|
| E.C. | 4.1.1.37 | |
| CSA | 1uro | |
| M-CSA | 1uro | |
| MACiE | ||
| CATH domain | Related DB codes (homologues) |
|---|
| Uniprot Enzyme Name | UniprotKB | Protein name | Synonyms | RefSeq | Pfam |
|---|---|---|---|---|
| P06132 |
Uroporphyrinogen decarboxylase
|
UPD
URO-D EC 4.1.1.37 |
NP_000365.3
(Protein)
NM_000374.4 (DNA/RNA sequence) |
PF01208
(URO-D)
[Graphical View] |
| KEGG enzyme name |
|---|
|
uroporphyrinogen decarboxylase
uroporphyrinogen III decarboxylase porphyrinogen carboxy-lyase porphyrinogen decarboxylase uroporphyrinogen-III carboxy-lyase |
| UniprotKB: Accession Number | Entry name | Activity | Subunit | Subcellular location | Cofactor |
|---|---|---|---|---|---|
| P06132 | DCUP_HUMAN | Uroporphyrinogen III = coproporphyrinogen + 4 CO(2). | Homodimer. | Cytoplasm. |
| KEGG Pathways | Map code | Pathways | E.C. |
|---|---|---|
| MAP00860 | Porphyrin and chlorophyll metabolism |
| Compound table | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Substrates | Products | Intermediates | |||||||
| KEGG-id | C01051 | C00011 | C03263 | ||||||
| E.C. | |||||||||
| Compound | Uroporphyrinogen III | CO2 | Coproporphyrinogen III | ||||||
| Type | aromatic ring (with nitrogen atoms),carboxyl group | others | aromatic ring (with nitrogen atoms),carboxyl group | ||||||
| ChEBI |
15437 15437 |
16526 16526 |
15439 15439 |
||||||
| PubChem |
1179 1179 |
280 280 |
321 321 |
||||||
| 1uroA |
|
|
|
|
|
Unbound | Unbound | Unbound | |
| 1jphA |
|
|
|
|
|
Unbound | Unbound | Unbound | |
| 1jpiA |
|
|
|
|
|
Unbound | Unbound | Unbound | |
| 1jpkA |
|
|
|
|
|
Unbound | Unbound | Unbound | |
| Reference for Active-site residues | ||
|---|---|---|
| resource | references | E.C. |
| literature;[8] | ||
| Active-site residues | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| PDB | Catalytic residues | Cofactor-binding residues | Modified residues | Main-chain involved in catalysis | Comment | |||||
| 1uroA |
|
|
|
|
|
ASP 86 | ||||
| 1jphA |
|
|
|
|
|
ASP 86 | mutant I260T | |||
| 1jpiA |
|
|
|
|
|
ASP 86 | mutant F232L | |||
| 1jpkA |
|
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|
|
ASP 86 | mutant G156D | |||
| References for Catalytic Mechanism | ||
|---|---|---|
| References | Sections | No. of steps in catalysis |
|
[1]
|
Scheme 11, p.27-28 | 4 |
|
[5]
|
Scheme 3, p.134-137 | 3 |
|
[8]
|
p.2467-2468 | |
| References | |
|---|---|
| [1] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 3895052 |
| Journal | Nat Prod Rep |
| Year | 1985 |
| Volume | 2 |
| Pages | 19-47 |
| Authors | Leeper FJ |
| Title |
The biosynthesis of porphyrins, |
| Related PDB | |
| Related UniProtKB | |
| [2] | |
| Resource | |
| Comments | VARIANT PCT GLU-281. |
| Medline ID | 87042763 |
| PubMed ID | 3775362 |
| Journal | Science |
| Year | 1986 |
| Volume | 234 |
| Pages | 732-4 |
| Authors | de Verneuil H, Grandchamp B, Beaumont C, Picat C, Nordmann Y |
| Title | Uroporphyrinogen decarboxylase structural mutant (Gly281----Glu) in a case of porphyria. |
| Related PDB | |
| Related UniProtKB | P06132 |
| [3] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 2180703 |
| Journal | Eur J Biochem |
| Year | 1990 |
| Volume | 188 |
| Pages | 393-403 |
| Authors | Felix F, Brouillet N |
| Title |
Purification and properties of uroporphyrinogen decarboxylase from Saccharomyces cerevisiae. |
| Related PDB | |
| Related UniProtKB | |
| [4] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 1576986 |
| Journal | Eur J Biochem |
| Year | 1992 |
| Volume | 205 |
| Pages | 1011-6 |
| Authors | Garey JR, Labbe-Bois R, Chelstowska A, Rytka J, Harrison L, Kushner J, Labbe P |
| Title |
Uroporphyrinogen decarboxylase in Saccharomyces cerevisiae. |
| Related PDB | |
| Related UniProtKB | |
| [5] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 7842850 |
| Journal | Ciba Found Symp |
| Year | 1994 |
| Volume | 180 |
| Pages | 131-51; discussion 152-5 |
| Authors | Akhtar M |
| Title | The modification of acetate and propionate side chains during the biosynthesis of haem and chlorophylls: mechanistic and stereochemical studies. |
| Related PDB | |
| Related UniProtKB | |
| [6] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 9194196 |
| Journal | Protein Sci |
| Year | 1997 |
| Volume | 6 |
| Pages | 1343-6 |
| Authors | Phillips JD, Whitby FG, Kushner JP, Hill CP |
| Title | Characterization and crystallization of human uroporphyrinogen decarboxylase. |
| Related PDB | |
| Related UniProtKB | |
| [7] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 9761933 |
| Journal | Acta Crystallogr D Biol Crystallogr |
| Year | 1998 |
| Volume | 54 |
| Pages | 476-8 |
| Authors | Laterriere M, d'Estaintot BL, Dautant A, Precigoux G, Hombrados I, De Verneuil H |
| Title |
Expression, |
| Related PDB | |
| Related UniProtKB | |
| [8] | |
| Resource | |
| Comments | X-RAY CRYSTALLOGRAPHY (1.8 ANGSTROMS). |
| Medline ID | 98232492 |
| PubMed ID | 9564029 |
| Journal | EMBO J |
| Year | 1998 |
| Volume | 17 |
| Pages | 2463-71 |
| Authors | Whitby FG, Phillips JD, Kushner JP, Hill CP |
| Title | Crystal structure of human uroporphyrinogen decarboxylase. |
| Related PDB | 1uro |
| Related UniProtKB | P06132 |
| [9] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 11719352 |
| Journal | Blood |
| Year | 2001 |
| Volume | 98 |
| Pages | 3179-85 |
| Authors | Phillips JD, Parker TL, Schubert HL, Whitby FG, Hill CP, Kushner JP |
| Title | Functional consequences of naturally occurring mutations in human uroporphyrinogen decarboxylase. |
| Related PDB | 1jph 1jpi 1jpk |
| Related UniProtKB | |
| Comments |
|---|
| Created | Updated |
|---|---|
| 2004-03-30 | 2009-02-26 |