3SBY,3SC0,3SBZ,3SOM


Conserved Protein Domain Family
MMACHC-like
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cd12959: MMACHC-like 
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Methylmalonic aciduria and homocystinuria type C protein and similar proteins
MMACHC, also called CblC, is involved in the intracellular processing of vitamin B12 by catalyzing two reactions: the reductive decyanation of cyanocobalamin in the presence of a flavoprotein oxidoreductase and the dealkylation of alkylcobalamins through the nucleophilic displacement of the alkyl group by glutathione. Mutations in MMACHC cause combined methylmalonic acidemia/aciduria and homocystinuria (CblC type), the most common inherited disorder of cobalamin metabolism. The structure of MMACHC reveals it to be the most divergent member of the NADPH-dependent flavin reductase family that can use FMN or FAD to catalyze reductive decyanation; it is also the first enzyme with glutathione transferase (GST) activity that is unrelated to the GST superfamily in structure and sequence.
Links
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Statistics
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PSSM-Id: 214022
Aligned: 27 rows
Threshold Bit Score: 175.925
Created: 18-Oct-2012
Updated: 2-Oct-2020
Structure
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Program:
Drawing:
Aligned Rows:
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Conserved site includes 23 residues -Click on image for an interactive view with Cn3D
Feature 1:cobalamin binding site [chemical binding site]
Evidence:
  • Structure:3SC0: Human MMACHC binds methylcobalamin, contacts at 4A.
  • Structure:3SOM: Human MMACHC binds cobalamin, contacts at 4A.
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Sequence Alignment
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Format: Row Display: Color Bits: Type Selection: 
Feature 1                                    ###                                                 
3SBY_A         7 ELKQKIEDTLCp-fGFEVYPFQVAWYNELLPPAFHLPLPGp----TXAFLVLSTPaXFDRALKPFLQs------------ 69  human
3SC0_A        10 ELKQKIEDTLCp-fGFEVYPFQVAWYNELLPPAFHLPLPGp----TLAFLVLSTPaMFDRALKPFLQs------------ 72  human
ADY47961      10 DVMDKLRRLFPdqeGFELHPFKVGSYNSVAGPCFQLPYKEd----TLGIILLNTPhFFETTFKKWIQmkkkpe------- 78  pig roundworm
ADY48351       6 KVLGHLCELLPetdGFECHRFKVGSYNALVASEFQLAYEAd----VMAVVVLNTPsFLETTFKHWLQsqkgeh------- 74  pig roundworm
ADY47105      70 KIFDTLHELLAecdGFEWHHFKVGSYNAVVQKGVQLRFDDd----VMAVVVLNTPsFFENTYKKWLQkqyksg------- 138 pig roundworm
ADY45152      62 IIYRALDKQLAqrdGFEWYRFKVGSHNAIVQENVRLKFEDd----VLGVLVLNTPsFFETTFKEWLRqhcnpg------- 130 pig roundworm
ACO15390       4 DIQGILEEHLGprqGFETHPFRIGDYNAKVSSKFSLPFEGd----SLGFALISTPsMFEKTFLPYLIsss---------- 69  Caligus clemensi
GAA53390      14 RTIQCLRGQLCp-fGFELAPFLVGWYNDIVSARLKIDTVTapfddCVALCIISAPhMFEDSFLHHLFdwfkgdevtslan 92  Clonorchis sine...
NP_001022524  10 SIKRVVDQKLSsheGFESHMFKIGSYNEAVGESSPFALPYdd--sTMALLILSTPdMFDVAFRKWVVqktmd-------- 79  nematode
XP_644271      3 QLIQSLRKDFKekgFDIVEPFRVNDYNGAAIHKLNNFGIEd----STGIIIGCTK-NFWPHFEQYIK------------- 64  Dictyostelium d...

Feature 1                                                                    #         # #####  #
3SBY_A        70 ------------CHLRXLTDPVDQCXAYHLGRVRESl--------------pELQIEIIADYEXHp-NRRPKILAQTAAH 122 human
3SC0_A        73 ------------CHLRMLTDPVDQCVAYHLGRVRESl--------------pELQIEIIADYEVHp-NRRPKILAQTAAH 125 human
ADY47961      79 -------etledFAKHFPSGPLSAFFNERFEEAIQMf--------------gAQEVVVIHDSEVLp-NRRPKVLMTTCGH 136 pig roundworm
ADY48351      75 -------ealneLIAKFGANPLQAYFIRKFGQVIKDl--------------rPVEARVIHDFDFNn-NCTPKVLLTTCGM 132 pig roundworm
ADY47105     139 -------esmetFMKKIGSNPIRSFFLSRFAQIENRl--------------lPIRAVSICDFEPLh-DRLPLVSLPVCGH 196 pig roundworm
ADY45152     131 -------egvddVKTKVGPHPVETFFNWKFMQIEMHl--------------sPIRVVSICDFEPVr-HRLPAVSLPACGH 188 pig roundworm
ACO15390      70 ---------tniEEASSPMDPLDECMRTTVNKVIHHhlig--------addiRHPILFLHDFEIHp-NRRPKILVQTAGH 131 Caligus clemensi
GAA53390      93 alksikpnlngcVALPGPNDPLDWSVFLRIHDATKAllgdlkvqlppedyqkLTPLRFIPDYAMKpvVRLPYIHVQCAGH 172 Clonorchis sine...
NP_001022524  80 -------fgsfdEVCEMVSSPIQSFLEDRLEIMSEKlr------------kvEENFEILHDYSMTp-QRRPKILMQTCGH 139 nematode
XP_644271     65 ------------RINNVPKDPMNCFVIDTIEGVVENnqf-----------ikPYEFEIRYDFNSP--SSGKYVHIQTCGH 119 Dictyostelium d...

Feature 1              # #                       # ##        ###                                 
3SBY_A       123 VAGAAYYYQrqdvead---------pwgnqriSGVCIHPRFGGWFAIRGVXLLPGi-eVPDLPPRKPHDCVPTra----- 187 human
3SC0_A       126 VAGAAYYYQrqdvead---------pwgnqriSGVCIHPRFGGWFAIRGVVLLPGi-eVPDLPPRKPHDCVPTra----- 190 human
ADY47961     137 VAGAAFFYHppeealgtan----sassakrrrAGVSLHPKYGGYFAFRGVFVFLNvrlPSDFKEERAPMLLDTve----- 207 pig roundworm
ADY48351     133 VSGAAYFYRplesgksrid----pvthvekrsMGLSLHPKFGGHFGFRAVFIFPRvhlPADFKEKTAPMRLDTpe----- 203 pig roundworm
ADY47105     197 ISGAAYFYRvkeatdpi--------tgkkklhMGLSLHPKYGGHFSFRGVIVFPDvrlPATYKEKLPLKTLDTee----- 263 pig roundworm
ADY45152     189 VSGAAYFYHpnhpvt-----------gekiypQGLSLHPKYGGYFAFRGVIIFPDvhlAATFKEPQPLKTLDTda----- 252 pig roundworm
ACO15390     132 VSGSVRLYQap-------------------gyNPVCLHPLYGGWFALRGALFFPNi--QYDDSPKEPPNILQSke----- 185 Caligus clemensi
GAA53390     173 VSGIAYFHRdnpih-------------ggsstAGCSLHPRFGGWFGFRGAIVFPNl-rCPTLPRPVPVSSLPPghppf-p 237 Clonorchis sine...
NP_001022524 140 VAGAAFYYQpchfqedgvtwppagrmgpnlkfIGLSLHPIYGGHFAFRSVLIFPNv-kIPEFCEKEPRPILTAse----- 213 nematode
XP_644271    120 FAGVASYDKd----------------------VMWSIHPTYGLWFVFRSCIIIKIn-yQGDSPNLPPTNLLTQfekdemk 176 Dictyostelium d...

Feature 1                  #   #    ##                           
3SBY_A       188 --dRIALLEGFNFHWRDWTYRDAvt---pqERYSEEQKAYFSTPPAQR 230 human
3SC0_A       191 --dRIALLEGFNFHWRDWTYRDAvt---pqERYSEEQKAYFSTPPAQR 233 human
ADY47961     208 --kQEEAITLFNTHWWDGRYRDCgn---pvEKYSDLQLKYFSTMPDKR 250 pig roundworm
ADY48351     204 --kQKEALDLFNNSWKDGRFRDCgd---plERYSELQLKYFSTPPMAR 246 pig roundworm
ADY47105     264 --kIDEALELFNEHYYDNRYRNCgn---pvETYSELQLKYFSTPPEQR 306 pig roundworm
ADY45152     253 --kVLEALELFNKHWMDNRYRDCgd---piEKYSALQLKYFNTVSRRR 295 pig roundworm
ACO15390     186 --eIHNLLELFNDHWRDARYRDVip---vkEKYSPLQRLYFETLPVDR 228 Caligus clemensi
GAA53390     238 pesVTLLLDSYRKRWRENEWRNLglpkdstNTYSPAARCFFNTPPEKR 285 Clonorchis sinensis
NP_001022524 214 --dVRTALEKFNYNWKDSGFRDFgn---ptRRYSTTQMEFFGRPVAER 256 nematode
XP_644271    177 rftQIAIDEGWINLETRLKIRDAcpi-greFRYEGNMFDYFYPLKRSS 223 Dictyostelium discoideum AX4

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