| General information | | DisProt: | DP00308 | | Name: | Uroporphyrinogen decarboxylase | | Synonym(s): | DCUP_HUMAN
URO-D
UPD
EC 4.1.1.37
| | First appeared in release: | Release 3.0 (02/17/2006) | | UniProt: | P06132 | | UniGene: | Hs.78601 | | SwissProt: | DCUP_HUMAN | | TrEMBL: | | | NCBI (GI): | 2507533 | | Source organism: | Homo sapiens (Human) | | Sequence length: | 367 | | Percent disordered: | 8% | | Homologues: | |
| Native sequence |
10 20 30 40 50 60
| | | | | |
MEANGLGPQG FPELKNDTFL RAAWGEETDY TPVWCMRQAG RYLPEFRETR AAQDFFSTCR - 60
SPEACCELTL QPLRRFPLDA AIIFSDILVV PQALGMEVTM VPGKGPSFPE PLREEQDLER - 120
LRDPEVVASE LGYVFQAITL TRQRLAGRVP LIGFAGAPWT LMTYMVEGGG SSTMAQAKRW - 180
LYQRPQASHQ LLRILTDALV PYLVGQVVAG AQALQLFESH AGHLGPQLFN KFALPYIRDV - 240
AKQVKARLRE AGLAPVPMII FAKDGHFALE ELAQAGYEVV GLDWTVAPKK ARECVGKTVT - 300
LQGNLDPCAL YASEEEIGQL VKQMLDDFGP HRYIANLGHG LYPDMDPEHV GAFVDAVHKH - 360
SRLLRQN
|
| Functional narrative |
Catalyzes the decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III.
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| Map of ordered and disordered regions |
Note: 'Mouse' over a region to see the start and stop residues. Click on a region to see detailed information.
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| Region 1 | | Type: | Disordered | | Name: | | | Location: | 1 - 9 | | Length: | 9 | | Region sequence: |
MEANGLGPQ | | Modification type: | Engineered
Mutant
| | PDB: | 1JPH:A, 1JPI:A, 1JPK:A | | Structural/functional type: | | | Functional classes: | | | Functional subclasses: | | Detection methods:
- X-ray crystallography (100 K; pH: 7; Beta-mercaptoethanol 1 mM; Glycerol 10 %; Tris 50 mM)
- X-ray crystallography (Beta-ME 1 mM; MES 100 mM; MPD (16-24% ); Tris-HCl (pH 7.5) 50 mM)
| References:
- Phillips JD, Parker TL, Schubert HL, Whitby FG, Hill CP, Kushner JP. "Functional consequences of naturally occurring mutations in human uroporphyrinogen decarboxylase." Blood. 2001; 98(12): 3179-85. PubMed: 11719352
- Whitby FG, Phillips JD, Kushner JP, Hill CP. "Crystal structure of human uroporphyrinogen decarboxylase." Embo J. 1998; 17(9): 2463-71. PubMed: 9564029
| Comments:This disordered region contains the first 9 amino acids of the SwissProt sequence, and does not include the N-terminal tag as found in the PDB file and both references.
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| Region 2 | | Type: | Disordered | | Name: | | | Location: | 366 - 367 | | Length: | 2 | | Region sequence: |
QN | | Modification type: | Engineered
Mutant
| | PDB: | 1JPH:A, 1JPI:A, 1JPK:A | | Structural/functional type: | | | Functional classes: | | | Functional subclasses: | | Detection methods:
- X-ray crystallography (100 K; pH: 7; Beta-mercaptoethanol 1 mM; Glycerol 10 %; Tris 50 mM)
- X-ray crystallography (Beta-ME 1 mM; glycerol 10 %; MES 100 mM; MPD (16-24%); Tris-HCl (pH 7.5) 50 mM)
| References:
- Phillips JD, Parker TL, Schubert HL, Whitby FG, Hill CP, Kushner JP. "Functional consequences of naturally occurring mutations in human uroporphyrinogen decarboxylase." Blood. 2001; 98(12): 3179-85. PubMed: 11719352
- Whitby FG, Phillips JD, Kushner JP, Hill CP. "Crystal structure of human uroporphyrinogen decarboxylase." Embo J. 1998; 17(9): 2463-71. PubMed: 9564029
| Comments:
|
| Region 3 | | Type: | Disordered | | Name: | | | Location: | 58 - 70 | | Length: | 13 | | Region sequence: |
TCRSPEACCELTL | | Modification type: | Mutant
| | PDB: | | | Structural/functional type: | | | Functional classes: | | | Functional subclasses: | | Detection methods:
- X-ray crystallography (100 K; pH: 7; Beta-mercaptoethanol 1 mM; Glycerol 10 %; Tris 50 mM)
| References:
- Phillips JD, Parker TL, Schubert HL, Whitby FG, Hill CP, Kushner JP. "Functional consequences of naturally occurring mutations in human uroporphyrinogen decarboxylase." Blood. 2001; 98(12): 3179-85. PubMed: 11719352
| Comments:
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| Region 4 | | Type: | Disordered | | Name: | | | Location: | 100 - 105 | | Length: | 6 | | Region sequence: |
MVPGKG | | Modification type: | Engineered
| | PDB: | | | Structural/functional type: | | | Functional classes: | | | Functional subclasses: | | Detection methods:
- X-ray crystallography (Beta-ME 1 mM; citrate (pH 5.8-6.2, 1.2-1.7 M); glycerol 10 %; Tris-HCl (pH 7.5) 50 mM)
| References:
- Whitby FG, Phillips JD, Kushner JP, Hill CP. "Crystal structure of human uroporphyrinogen decarboxylase." Embo J. 1998; 17(9): 2463-71. PubMed: 9564029
| Comments:
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