3D8T


Conserved Protein Domain Family
HEM4
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pfam02602: HEM4 (this model, PSSM-Id:145641 is obsolete and has been replaced by 426866)
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Uroporphyrinogen-III synthase HemD
This family consists of uroporphyrinogen-III synthase HemD EC:4.2.1.75 also known as Hydroxymethylbilane hydrolyase (cyclizing) from eukaryotes, bacteria and archaea. This enzyme catalyses the reaction: Hydroxymethylbilane <=> uroporphyrinogen-III + H(2)O. Some members of this family are multi-functional proteins possessing other enzyme activities related to porphyrin biosynthesis, such pfam00590, however the aligned region corresponds with the uroporphyrinogen-III synthase EC:4.2.1.75 activity only. Uroporphyrinogen-III synthase is the fourth enzyme in the heme pathway. Mutant forms of the Uroporphyrinogen-III synthase gene cause congenital erythropoietic porphyria in humans a recessive inborn error of metabolism also known as Gunther disease.
Links
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Statistics
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PSSM-Id: 145641
Aligned: 138 rows
Threshold Bit Score: -1
Created: 5-Apr-2011
Updated: 12-Apr-2011
Structure
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Program:
Drawing:
Aligned Rows:
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PubMed ReferencesClick to see Conserved Features Help
Sequence Alignment
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Format: Row Display: Color Bits: Type Selection: 
3D8T_B        45 EEFKALA--EKLG-FTPLLFPVQATEKvpvpeyrdqvrelaQGV---------DLFLATTGVGVRDlle--agkALGLDL 110 Thermus thermop...
Q6C2A4        14 DFYQE-LfvEQKG-HNAVFVPLLQHSPvgiesl---kthiaNEVdg------iDAVIVTSHRAVEI--------LGEYAS 74  Yarrowia lipoly...
Q9W3B3        19 DVYAETL--EKHD-FNPVFVPTLSFGFkn----------ldELRaklqnpdkyAGIIFTSPRCVEAvae---slnLGELP 82  fruit fly
Q17JM0        20 EAYPALL--RKHG-FEPIFVPTLDFSFrldil----rdrllSPY----------SIRYHSPRAITAvrd---aldGNRLK 79  yellow fever mo...
Q2S2A7        34 DRYRAAL--REAG-LRAVCEPVLAFTFprqaql---rdrlaAPDp-------yAGLLATSPRATVAleq--vfaSHEALA 98  Salinibacter ru...
Q5DB19        19 DPYVQIL--AKSG-FNVNLLETLDFEYnt-----------sNLAeyknwrnnhSCIVFSSPRAVIS--------YVKAGL 76  Schistosoma jap...
Q2ULI2        25 DSYEEYF--SAHS-YNPTFIPVLEHNFhtpnlttvkqlfqsGALnpgp-grkyGGLIFTSQRAVEGfatilndiGESTKQ 100 Aspergillus oryzae
XP_001635247  21 DRYHMEL--RSHG-FQCTSIPVLSFEFenldkl---ekmlsEPTe-------yGGIILTSARGVEAme------------ 75  starlet sea ane...
EDK41462      20 DPYHDVF--TDAG-FEPTFIPLLKHGHvdre-------ksiEYFtgee-flsiPVFIITSQRAVEClnec-lehVSSEIK 87  Pichia guillier...
YP_001295118  18 SPYFEL---QNKHkVKIDFRPFIHVEGvlakd----vrtqkIDLnn------fTAIILTSKNSVDHffr----vaEEMRY 80  Flavobacterium ...

3D8T_B       111 EGPLAKAFRlARGAKAARALKE---Ag-LPP-------HAVGDGTSKSLLPLLPQ----------GRgvaALQLYG-KPL 168 Thermus thermop...
Q6C2A4        75 PELLCKPAY-TVGPATARCLRE---Lg-FKDv-----rGEE-TGNGAALVPQLLsd-------gyQR---FAFYAGkVRR 133 Yarrowia lipoly...
Q9W3B3        83 GGWKMLHNY-AVGEVTHNLALS---T--LDQlf----tHGKQTGNARALGDYIVdtf------dgSRalpLLLPCGnLAT 146 fruit fly
Q17JM0        80 DDWKPLENY-SVGETSSELIQR---T--LELd-----tKGQHSGNASNLADFIKTdly-----nkTVtlpFLFPCGnLKQ 143 yellow fever mo...
Q2S2A7        99 DGWADRLAY-AVGPKTAARLRA---Lg-LRA-------RGDDTGTADALAARIVKdd------pdAP---LLFLSGnRRR 157 Salinibacter ru...
Q5DB19        77 TGNENDLCF-VVGPSTELQAKK---Ag-FSP-------KGSDSGDAVKLADFILKhy------ssKLdkpIFFPSGqVHL 138 Schistosoma jap...
Q2ULI2       101 TSSQSLILY-TVGPATSRSLTS---I--RDDhlphatiLGSETGNGENLAHFILShynplydsqdGPkppLLFLVGeQRR 174 Aspergillus oryzae
XP_001635247  76 ---------kCIEEKISLSAKK---KlgLES-------VGHQAGSAEMLAPMILeg-------ahPQtkpFLFPCGnLRK 129 starlet sea ane...
EDK41462      88 TVIHEKTVY-TVGPATTEVLRE---Ag-FTNi-----kGGSDAGNGSVLSDIIIKdvqe---ggpSK---MIFLTGeIRK 151 Pichia guillier...
YP_001295118  81 KVPEDLKYF-CQSEAVAFYLQKyvvYr-KRKi-----yVGQKDFVD---MSTLFKky------kdEK---YLLPSSdQLN 141 Flavobacterium ...

3D8T_B       169 PLLENAL-A-----ERGYRVLPLMPYRH------LPDPEGILRL--EEAvlr----gevDALAFVAAIQVEFLFEGAKdp 230 Thermus thermop...
Q6C2A4       134 DTIPNKL-R-----EAGRSLVEYTVYETv----dLDDIAGRF-----EKavgalpdakhNWICFFAPSGTAQIVEYLKk- 197 Yarrowia lipoly...
Q9W3B3       147 DTLLSKL-A-----ENGFSVDACEVYETrchpelGANVERALEIygESI----------EFLAFFSPSGVNCAQQYFTsr 210 fruit fly
Q17JM0       144 DVLQNKL-S-----EYGYSLDCVEVYETvphrdlERNLVELFREgaEQP----------GYLLFFSPSGMNYCASVFErh 207 yellow fever mo...
Q2S2A7       158 ETLPEAL-R-----AAEVRFDELVVYET--------HARTDL-----DVpar----graSWLVFFSPSGLEAVTRAEGv- 213 Salinibacter ru...
Q5DB19       139 DTLPKVL-E-----NAGKKVNTVVAYSSventqlHEKLRLNLCEgnPIP----------ECLVYFSPSGVSLTEKILIte 202 Schistosoma jap...
Q2ULI2       175 DIIPKTLmAgslppEQRIGVDELVVYETgvmegfEKSFGEAVRA--SEEflggg-veraVWVVVFSPTGCDAMVRVLKgl 251 Aspergillus oryzae
XP_001635247 130 ETIPTEM-Q-----NAGVPLKGLQVYKTc----kHTELAQNL-----TShvee--kgvpSVIVFFSPSGVQFVLSLLTei 192 starlet sea ane...
EDK41462     152 DIIPRKL-K-----QAKIDLIEKVIYKTe----pRDDIVSNFHSssKDV----------RWVVFFSPQGTVPIVEHLQga 211 Pichia guillier...
YP_001295118 142 ADITQTL-N-----NLKVDWIAGTFYKT---------VMSDL-----SDlkd----vyyDVLAFFSPTGIKSLFKNFPn- 196 Flavobacterium ...

3D8T_B       231 kalreaLNTRVKALAVGRVTADALR-EWGVKPfYVDETERLGSl--LQ 275 Thermus thermophilus
Q6C2A4       198 -----nPAHGFKIASIGPTTLDYLQ-KNDIKVdVTAKQPDPEHf--LE 237 Yarrowia lipolytica
Q9W3B3       211 ----qlSMDKWKLVAIGPSTRRALE-SLGQKVyCTAERPTVEHl--VK 251 fruit fly
Q17JM0       208 ----klDINGCRIVAIGPSTKKAIE-NKGYAVhRTAEKPSPEYv--VS 248 yellow fever mosquito
Q2S2A7       214 ------DVQAHRLAAIGPTTAEALE-GDGHAVeAVADEPSPDGl--VA 252 Salinibacter ruber DSM 13855
Q5DB19       203 i---kpHRPNIKLVAMGRATASRLK-ELGLTIsAVASSPKPESl--LA 244 Schistosoma japonicum
Q2ULI2       252 ----gdGERRVFVATIGPTTRDHLKnKYGFEAdVCAEKPSQEG---VG 292 Aspergillus oryzae
XP_001635247 193 ----fgDFSALKLVAIGATTSAAMI-KHGLSVaAVAKQPSPKSl--AE 233 starlet sea anemone
EDK41462     212 e---rsSAMGPFVASIGPTTEEYLQ-SYSIGIdAVASKPEAKSl--LA 253 Pichia guilliermondii ATCC 6260
YP_001295118 197 -----fEQKDTRIAVFGSTTQKEAL-EHGLRIdILAPTPETPSmtmAL 238 Flavobacterium psychrophilum JIP02/86
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