1WD7


Conserved Protein Domain Family
HEM4

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pfam02602: HEM4 
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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 catalyzes 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 as HemD with 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.
Statistics
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PSSM-Id: 396937
Aligned: 76 rows
Threshold Bit Score: 93.5413
Threshold Setting Gi: 146400828
Created: 20-May-2020
Updated: 7-Aug-2020
Structure
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Program:
Drawing:
Aligned Rows:
PubMed ReferencesClick to see Conserved Features Help

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
1WD7_B        21 AFKALA--EKLGFTPLLFPVQATEKvpvPEYRDQVRALA-Q------GVDLFLATTGVGVRDLLEAGKALGLDLEGPLAK 91  Thermus thermop...
Q5UY50        21 DAVELI--ESLGADPVPDPMLAIEPa-tADDAEATGRQV-Tprs---daDYVVLTSKTGVEL-------AAEAGWDAGEA 86  Haloarcula mari...
WP_012186937   7 VIRAKGeeGIRGEGVIEVPVLKPIP------DQNALRRL-Eelc-srRWDVVVFMSTVAVEY-------TYSVVKDPCWV 71  Caldivirga maqu...
O26268        12 EAVELI--EGAGGRALVAPTLELKE----AHTESLREVC-Rra---dEWDLVIFTSQAAVESLFQLCREFAGKIRK--DC 79  Methanothermoba...
Q1D8Z7        21 ELCFLL--EDEGAEVLSVPLLELHP---PEDPRPLASAA-Ehi---qRYTWVVFASPTGVDALMEALR-EAGTQDRIQRV 90  Myxococcus xant...
Q3JDS0        28 KLSQAI--ELEGGQAVRFPTVEIGD-------SEVPAVVeEiigrldDFHWAVFVSANAVQRgv---pWVFARREMPANL 95  Nitrosococcus o...
Q5ZRY5        21 VLNKKI--IESGGIPIDCPTMEIMA--------SETNWI-NnlpdltSVNQAIFVSTNAVRYFMMELA--SKNINWPNSI 87  Legionella pneu...
Q5F9N4        19 ddvEVC--RRAGWQAEVLSPIEIET-----DEASLKRLP-Emy---aRADAVFWVSPAAVETAVPYLN-------LSDGI 80  Neisseria gonor...
Q65VX8        14 QLVDML--NKSGVAALHLPFFNITA------GRELNDLP-NkfnqlkPNDYVVAVSQSAVDFAAETLQ--NTGFHWRTDL 82  [Mannheimia] su...
Q7VRM3        14 DLVDRL--VSIGKSAYHLPLISFST------GKTLFSIK-KylnflhTGDFLFIVSQHAVKYAHIQLL--SSGTQWPHKI 82  Candidatus Bloc...
1WD7_B        92 AFRlARGAKAARALKEAg-LPPHAVG--DGTSkSL--LPLLP-QGRG-VAALQLYGkp-lPLLENALAERGYRVLPLMPY 163 Thermus thermop...
Q5UY50        87 TVC-AIGDSTAEALHEAg-YGVDIIPa-EYSStGL--VETLSgAVAG-ARVEVARSdhgsAVLTDGLEAAGAYVHETVLY 160 Haloarcula mari...
WP_012186937  72 RCM-AVGPSTAAAVRRL--YNNECLIpsEYSSmGL--LRMLRgFNGS---ILVIRSmeysNSLFNEL----GGVTEIGLY 139 Caldivirga maqu...
O26268        80 LVA-VIGPRTARVAGEHg-LRVDIVPe-DYTAeGL--LDALTgLNIEgWKVALPRTlsarKVLPRGLEMMGAEVLVAEAY 154 Methanothermoba...
Q1D8Z7        91 KVA-TVGPRTTRAAEGYg-LVVVAEPs-EGTGaAL--LNLIKdALQPgDEVLLPAAeegrRELEDGLREYGLLVTRVTAY 165 Myxococcus xant...
Q3JDS0        96 KIA-VVGRATAQALGNFg-LYPRLCPsyGYNSeALlaMDELQ-DMKG-QQVVLFRAsggrELLAKTLTKRGAKVEYAEVY 171 Nitrosococcus o...
Q5ZRY5        88 RVT-AIGKGTSRVLNEFn-IQVSDVPelPDSe-HLltLNSLQ-QIRN-QTILLVKGeggrQLIEEYLMLKGAKLCLLSVY 162 Legionella pneu...
Q5F9N4        81 KAHiAVGQGSRRALARYa-GAGVFAPedGNDSeAVlrLPVWNsLPEG-ARVLSVRGhggrDFLMNALQEKGFRTEVAEVY 158 Neisseria gonor...
Q65VX8        83 QYF-TVGQQTALHFASLs-EQPVHYPflSENSeGMlaLAQMQ-NLKG-KNVLLLRGnsgrELFPQQVLARGGDIDILECY 158 [Mannheimia] su...
Q7VRM3        83 TYY-SIGHKTSSKMYSLsgILSK-YPqnEETSeNLlqFPELIyNISG-RRALILKGnnglTTLQHTLQKRGALVLCCECY 159 Candidatus Bloc...
1WD7_B       164 RH--LPDPEGILRLEEALLRGEV----DALAFVAAIQVEFLFEGAKDPKal--reALNTRVKALAVGRVTADALREWGVK 235 Thermus thermop...
Q5UY50       161 RL---VRPEGSGESAELAASGDL----DAALFTSSLTVEHFLDAAADLGlreaviDGLNEATVAAIGQPTRETAEDAGIS 233 Haloarcula mari...
WP_012186937 140 RL--SVNEDELSKLKALINEREAagspYALIITSPRVAREVSGILL----------NLRRALIVAIGPTTAKELSMLNIP 207 Caldivirga maqu...
O26268       155 RSglPEDTGPAEELIDGLLDGKV----DAVTFTSPLTVENLFKIAGNRRke--liEVLKRVKVAAIGPITLRKLEEHGIT 228 Methanothermoba...
Q1D8Z7       166 RA---TPAPLPPESLALLDASPP----DVVLFASPRTAEAFVEEAGRE--------RLATARVVAIGPTTASALERLGVP 230 Myxococcus xant...
Q3JDS0       172 RRclPSPS-VAANLRQVLVNVPV----DVVITTSKAVLENLCHLAGEPLl-----EKLRRTPLVVIGSRQVQQARELGFT 241 Nitrosococcus o...
Q5ZRY5       163 KRvmPAI--DQEFINSLWRDDLV----DIILLTSEQSIHNLFKMFSIEGq-----MWLQNKPCLVISDRLAKVASLFGIK 231 Legionella pneu...
Q5F9N4       159 FR------RHKPLNFQNFQTENI----AAAYITSTELVQSLFAQLPPQFs-----RFFKSLLYFTHHPRIAEALKREGVC 223 Neisseria gonor...
Q65VX8       159 QR--QPIDYDNVEQTSICKRAGI----QTIVVTSGELLNTLIQFVPENEy-----DWLRSCQLVVVSTRIENMARKFGWT 227 [Mannheimia] su...
Q7VRM3       160 TR--KPIQYNGKEQYNRMITLDI----EIIVVTTTDMLKQLYYLIPQSHr----tDWLITRQLVVTSNRLAEYAKSLGWS 229 Candidatus Bloc...
1WD7_B       236 PfYVDETERLGSlLQ 250 Thermus thermophilus
Q5UY50       234 VdVVPDQADFEAlAC 248 Haloarcula marismortui
WP_012186937 208 H-SIAKVYTIDGaVN 221 Caldivirga maquilingensis
O26268       229 A-VTPERYTVKDmIA 242 Methanothermobacter thermautotrophicus str. Delta H
Q1D8Z7       231 AaAVAERPTPESlVD 245 Myxococcus xanthus DK 1622
Q3JDS0       242 QiWVAQEPSDAAlVQ 256 Nitrosococcus oceani ATCC 19707
Q5ZRY5       232 KiLIS---HPERmIG 243 Legionella pneumophila subsp. pneumophila str. Philadelphia 1
Q5F9N4       224 S--VETVPTLEAals 236 Neisseria gonorrhoeae FA 1090
Q65VX8       228 DiVVSPKADNNTlLQ 242 [Mannheimia] succiniciproducens MBEL55E
Q7VRM3       230 DiIITKSANNTTiLR 244 Candidatus Blochmannia floridanus
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