2QLY,3W37,3LPO,3TON


Conserved Protein Domain Family
GH31_MGAM_SI_GAA

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cd06602: GH31_MGAM_SI_GAA 
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maltase-glucoamylase, sucrase-isomaltase, lysosomal acid alpha-glucosidase
This subgroup includes the following three closely related glycosyl hydrolase family 31 (GH31) enzymes: maltase-glucoamylase (MGAM), sucrase-isomaltase (SI), and lysosomal acid alpha-glucosidase (GAA), also known as acid-maltase. MGAM is one of the two enzymes responsible for catalyzing the last glucose-releasing step in starch digestion. SI is implicated in the digestion of dietary starch and major disaccharides such as sucrose and isomaltose, while GAA degrades glycogen in the lysosome, cleaving both alpha-1,4 and alpha-1,6 glucosidic linkages. MGAM and SI are anchored to small-intestinal brush-border epithelial cells. The absence of SI from the brush border membrane or its malfunction is associated with malabsorption disorders such as congenital sucrase-isomaltase deficiency (CSID). The domain architectures of MGAM and SI include two tandem GH31 catalytic domains, an N-terminal domain found near the membrane-bound end, and a C-terminal luminal domain. Both of the tandem GH31 domains of MGAM and SI are included in this family. The domain architecture of GAA includes an N-terminal TFF (trefoil factor family) domain in addition to the GH31 catalytic domain. Deficient GAA expression causes Pompe disease, an autosomal recessive genetic disorder also known as glycogen storage disease type II (GSDII).
Statistics
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PSSM-Id: 269888
Aligned: 116 rows
Threshold Bit Score: 429.624
Created: 11-Sep-2008
Updated: 2-Oct-2020
Structure
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Program:
Drawing:
Aligned Rows:
 
active sitecatalytic site
Conserved site includes 13 residues -Click on image for an interactive view with Cn3D
Feature 1:active site [active site]
Evidence:

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
Feature 1                          #                           ##                                 
2QLY_A        282 GRPALPSYWALGFHLSRYEYgTLDNMREVVERNRAaqlPYDVQHADIDYMDERRDFTYDSvDFKgf--pEFVNELH-NNG 358  human
3W37_A        312 GRPAPMPYWAFGFHQCRWGYrDVNEIETVVDKYAEariPLEVMWTDIDYMDAFKDFTLDPvHFPldkmqQFVTKLH-RNG 390  beet
CAK86633      252 GGWMLPPFWGFGFHQCRWGYkNSSVLIDVVQQYQKnliPIDIIWTDLDYMDDRQIFSVDKhKFPk----KDFQYLK-GLG 326  Paramecium te...
CAK94005      217 GGWALHPFWAMGYHHSRWPIkSSQKLKEYVYKHKEhdiPLDTIWSDIDYMNDRQIFSVDEmRFHk----NDFEEIQnQLG 292  Paramecium te...
XP_004037128  196 GGHILPPFWAFGFHQSRWGYsTSDKLIQVIKQYRRhgiPLDSIWSDIDFMINKQTFSVNFhLFSk----EFFQNFHeKYN 271  Ichthyophthir...
EAR98617      352 GKQALMPLWAYGFHQSRWGYqNIDEVKQIIAEYNFiefPVESIFIDIDYMINYNTFTVNQtIFPl----NNYSIMK-DIE 426  Tetrahymena t...
XP_646281     265 GTTHMPSYWSLGYHNCRWGYhSIAETAQVVANYSKyniPLETMWNDIDYMDSFRDFTTDPvNFAasdykTFIDGLH-ANN 343  Dictyostelium...
XP_004355852  278 GRTFMPSYWSLGWHQCRWGYtSIEETAQVVANYSLhgiPLETMWNDIDYMNAYMDFTLDPvNFNqtavrALIDQLH-ENN 356  Dictyostelium...
EFA78629      693 GTPYMPSYWSLGWHQCRWGYkSVNESKEVVLNYAKygiPLETMWNDIDYMDRYEDFTLDPvNYPesemtAYVDWLH-SNN 771  Polysphondyli...
XP_002676411  139 GTPYMPPFWTLGWHQCRYGYkSIDEVKTVYTKYTQygiPLDTMWNDIDYMDGYRDFTTDPiRYPkaevrQFVENLK-AKN 217  Naegleria gru...
Feature 1              #                                                   #                      
2QLY_A        359 QKLVIIVDPAIsnnss------sskpYGPYDRGSDMKiwvns----sdgvtPLIGEVWPGQtVFPDYTNPnCAVWWTKEF 428  human
3W37_A        391 QRYVPILDPGIntn----------ksYGTFIRGMQSNvfik------rdgnPYLGSVWPGPvYYPDFLDPaARSFWVDEI 454  beet
CAK86633      327 VRYIPLLDVAVgv------------kYGSEDEGYRKGteydvflyspytgyRFQGYVWPGDsYFPDFFHPnISKYWNEMH 394  Paramecium te...
CAK94005      293 VNYIPIIDVAVgv------------kYGKQDKGFREGinldiflrspntgqRFRGKVWPGSsYFPDFFHPnCSTYWRTMI 360  Paramecium te...
XP_004037128  272 INYIPIVDVAVgvhp--------fagDNALKKGIEMDifcks----pqtgkYFKGYVWPGDsYFPDFLHPnSSVYWQQMM 339  Ichthyophthir...
EAR98617      427 KRIVPILDVGIglenqt---lqvrqdALYYNVGVKDLth-----------kIYIGRVWPGYtLFPDFQKPiTKIFWQNYT 492  Tetrahymena t...
XP_646281     344 QHYIMIVDPGIsniepiyqshidlmeSGAYIKAGGTDg------------ePLVGSVWPGYvNFPDFLHPnGTEFWTQQF 411  Dictyostelium...
XP_004355852  357 QHYMMIVDPGIhnq----------qgYESYDSLVQSNaylkt-----tsgeQQVGWVWPGStIFPDFFHPnASQYWLEQF 421  Dictyostelium...
EFA78629      772 QHYIMIVDPGIhtn----------dtYEPYSQLTNIEgfika-----sdgtPFVGVVWPGStIFPDFFNPkTNIFWKNWL 836  Polysphondyli...
XP_002676411  218 QHYVVIIDPGIkfe----------qgYKPYDIGKQLNvfikk----sdgvtDIVNTVWPGYcVFPDFTNPkAIPYWENLV 283  Naegleria gru...
Feature 1                     # ##                                                                
2QLY_A        429 ELFHnqvEFDGIWIDMNEVSNFvdgsvs----------------------gcstnnLNNPPFTPril----dgyLFCKTL 482  human
3W37_A        455 KRFRdilPIDGIWIDMNEASNFitsap------------------------tpgstLDNPPYKInnsg--grvpINSKTI 508  beet
CAK86633      395 EHLYeqvEFDGLWVDMNEPANFcegecd----------------------wnklnnEIIFPYIPgq------ipLANKTL 446  Paramecium te...
CAK94005      361 KHLYqstNFSGLWIDMNEPTNFcdgecdlqqgrdkwn----stmdytdvnetyknnHIRFPYIPgv------spLEKMTL 430  Paramecium te...
XP_004037128  340 EELHskvNFSGIWIDMNEPANFcngectwidfnqqqsnkviyikqnlikikiddpeYINQKYEIpyqiggynydLNLKTL 419  Ichthyophthir...
EAR98617      493 FNFMkqnNISAFWLDMNEYANFchgiceelyqnyrq-------synldvelfveglAYQPGSKP----------LENMSL 555  Tetrahymena t...
XP_646281     412 QRFYetvQFDGVWIDMNEISNFcdgncfnntd---------------tpmpppqfdPNYPPYIPgg------vpLYSKTI 470  Dictyostelium...
XP_004355852  422 QAFRemvPFDGIWLDMNELANFcnacipwleegiaee-----leasqsesssdafdPNNPPYVPgt------tiIYFNSI 490  Dictyostelium...
EFA78629      837 QTFQetvAYDGVWIDMNEVSNFcngncngd------------------ngkmsgfdPNNPPYLPgg------isLDKHTI 892  Polysphondyli...
XP_002676411  284 AQFYseiPVSGLWLDMNEVSCFcngtctptkk---------------yqkvnnkfdPNNPPYVPgg------rqLDMKTL 342  Naegleria gru...
Feature 1                                                       #            #  #                 
2QLY_A        483 CMDavqh--wgkqYDIHNLYGYSMAVATAEAaktv-fpnkRSFILTRSTFAGSGKfaAHWLGDNTATWDDLRWSIPGVLE 559  human
3W37_A        509 PATamhyg-nvteYNAHNLYGFLESQATREAlvrt--sneRPFLLSRSTFAGSGKytAHWTGDNAARWDDLQYSIPTMLN 585  beet
CAK86633      447 PPHllhhg-qylhKDVHNLYGIMDSYYTYQAqkel--gkvQPFQITRSTFPGTGKyaQHWTGDNGASWDFLYLSLGQVFQ 523  Paramecium te...
CAK94005      431 PPNlfhyg-khlhKDVHNLYGLQESFETYQAqkei--gkpLPFIISRSTFPGSGHftQHWEGDNEASYTFLYLSVGSTMQ 507  Paramecium te...
XP_004037128  420 PYHllhyg-kyehKDVHNIYGLLDNHHTYNTliadkikkiYPFILTRSSFPGSGKyaFKWTGDNLSDWNFLRISIVSIVN 498  Ichthyophthir...
EAR98617      556 SLVsiheesgdleFNFHNINGYLHSQSTYKAvkye-mqnpLPFLISRSTIPGSGNyvSHWAGDNNSSYEFMAYSVSSLFN 634  Tetrahymena t...
XP_646281     471 NMTslqyn-ntlvYNSHSLYGYTEGLATQLAvqsi--lgtRSTIISRSTFPGTGAhfAHWLGDNKSTFTDMYYSIPGMLA 547  Dictyostelium...
XP_004355852  491 NMStvqyn-ntnyYDAKSLYGFMESMVTTDIakql--lnqRSTLISRSTFPGTGRnnGHWLGDNASEFVEMYWSIPGIIA 567  Dictyostelium...
EFA78629      893 NLTttqyg-nlsvFDTHNLYGYTESLATVDAviei--lqkRATVVTRSTFPGSGNhaAHWLGDNNSQYSDMYYSIPGILN 969  Polysphondyli...
XP_002676411  343 SMDavqh--isinYNTHSLYGLYEVNATVSVlqkv-tgnkRPFILTRSSYPGLGAisAKWLGDNEATWESMRNSISGMLA 419  Naegleria gru...
Feature 1                        #                        #                                       
2QLY_A        560 FNLFGIPMVGPDICGFALDTPEELCRRWMQLGAFYPFSRNHNGqgyKDQDPASFgadsLLLNSSRHYLNIRYTLlPYLYT 639  human
3W37_A        586 FGLFGMPMIGADICGFAESTTEELCRRWIQLGAFYPFSRDHSArdtTHQELYLWe---SVAASARTVLGLRYQLlPYYYT 662  beet
CAK86633      524 FQIFGIPMVGADVCGFMGDTNDKLCCRWIQLAFFYPFFRNHNNdlsKPQEFYNLg--fHVIQSAQKNIHLRYTLlKWFYS 601  Paramecium te...
CAK94005      508 FNIFGIPMVGADVCGFLDNTTPKLCARWVQLGSLYPFFRNHNNdraKDQEFYSLg--dDVLLAARRNVKLRYSIiKWYYS 585  Paramecium te...
XP_004037128  499 LGLYGMPFAGADVCGFMGNTQKELCQRWIQLSSLQPFMRNHNHdqaKEQEFYNLg--qQVENTAIKNLKLRYQIlKHYFM 576  Ichthyophthir...
EAR98617      635 FHIFGIDFTGSDICGFMGNTTQELCNRWAQLGSLYPFSRNHNHeklLSQEFFTF----DEFGGALFSFRTKYSLlKYYYT 710  Tetrahymena t...
XP_646281     548 MNMFGIPMVGADICGFNGDSNAELCGRWLQLGCFYPFTRNHNTflgAPQEPWVFg--qEVVDISIKAINGKLTLlPFYYT 625  Dictyostelium...
XP_004355852  568 MNLFGINLVGADICGFNGNTTVELCTRWTQLGVFYPFSRNHNAigmNSQEPYVLg--qQVIDATITGVNNKYTLlPYYYT 645  Dictyostelium...
EFA78629      970 MNMFGIPLVGADICGFSGNTTAELCGRWMQLGNFYPFSRNHNDktaNSQEPYVFn--dTVTSIAIAAIHTKYTLlPFYYT 1047 Polysphondyli...
XP_002676411  420 MQMYGVALIGADICGFIGNTTYELCARWTQMGSYYPFTRNHNDikaNDQEPYVFg--qEFVDMTKRVLANRYSLlNFYYT 497  Naegleria gru...
Feature 1               
2QLY_A        640 LFFRAH 645  human
3W37_A        663 LMYDAN 668  beet
CAK86633      602 VFIREQ 607  Paramecium tetraurelia strain d4-2
CAK94005      586 LFLRSN 591  Paramecium tetraurelia strain d4-2
XP_004037128  577 LFVKTN 582  Ichthyophthirius multifiliis
EAR98617      711 LTLNNT 716  Tetrahymena thermophila
XP_646281     626 LFHISH 631  Dictyostelium discoideum AX4
XP_004355852  646 LFHWSH 651  Dictyostelium fasciculatum
EFA78629     1048 LFYLSH 1053 Polysphondylium pallidum PN500
XP_002676411  498 QFYDVH 503  Naegleria gruberi strain NEG-M

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