Conserved Protein Domain Family
GT8_LARGE_C

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cd06431: GT8_LARGE_C 
LARGE catalytic domain has closest homology to GT8 glycosyltransferase involved in lipooligosaccharide synthesis.
The catalytic domain of LARGE is a putative glycosyltransferase. Mutations of LARGE in mouse and human cause dystroglycanopathies, a disease associated with hypoglycosylation of the membrane protein alpha-dystroglycan (alpha-DG) and consequent loss of extracellular ligand binding. LARGE needs to both physically interact with alpha-dystroglycan and function as a glycosyltransferase in order to stimulate alpha-dystroglycan hyperglycosylation. LARGE localizes to the Golgi apparatus and contains three conserved DxD motifs. While two of the motifs are indispensible for glycosylation function, one is important for localization of th eenzyme. LARGE was originally named because it covers approximately large trunck of genomic DNA, more than 600bp long. The predicted protein structure contains an N-terminal cytoplasmic domain, a transmembrane region, a coiled-coil motif, and two putative catalytic domains. This catalytic domain has closest homology to GT8 glycosyltransferase involved in lipooligosaccharide synthesis.
Statistics
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PSSM-Id: 133053
View PSSM: cd06431
Aligned: 8 rows
Threshold Bit Score: 459.247
Threshold Setting Gi: 92058691
Created: 11-Jun-2008
Updated: 2-Oct-2020
Structure
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Aligned Rows:
 
putative ligandmetal binding
Feature 1:putative ligand binding site
Evidence:
  • Comment:annotation is based on homology to other subgroups of the superfamily
  • Comment:Donor and acceptor binding seems to be cooperative; in some glycosyltransferases the nucleotide-sugar bind first, and in others, the acceptor

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
Feature 1             ###   ##                                                                   
AAU12251     139 IHIAIVCAg-yNASRDVVTLVKSVLFHRrnPLHFHIITDSIARKILADLFHTWmvpavhvNFYDADELkseVSWIPNKHY 217 zebrafish
O95461       138 IHVAIVCAg-yNASRDVVTLVKSVLFHRrnPLHFHLIADSIAEQILATLFQTWmvpavrvDFYNADELkseVSWIPNKHY 216 human
Q21389        42 IRLAYIIGgnfMTRLMFMQHFKSVLKYSdhFFRLHLITDENHRSDIHELMTSWnisncewFFHNLTEFekrVAWIPNSHY 121 nematode
NP_001004383 138 IHVAIVCAg-yNASRDVVTLVKSVLFHRrnPLHFHLIADAIAKQILATLFQTWmvpavriDFYDADELkseVSWIPNKHY 216 chicken
AAH60410     107 IHVAIVCAg-hNSSRDVVTLVKSILFHRrnPLHLHLITDDVALRVLGNLFNTWmvpslqiSFYNASELkpdVAWIPNKHY 185 African clawed ...
XP_001176040   5 IHVSIVCAg-hNASRDVIILIKSILFYRknPLHFHFISDEMGQLILSTLFKTWdvpgvkvSFYLAENYkshVEWIPNKHY 83  purple urchin
XP_624139     94 VHIAMVCAg-yNSTFALVTVVKSVLFYRtkPLHFHLLVDEIAKRTLTTVFQTWdlphvnlTYYKAEKWvpkVSWIPNKHY 172 honey bee
XP_001640724 120 IHIAVVCAg-hSASRQAVTLIKSLLFYRhnPLHFHFVSDSVAELILGTLFRTWgvpavqtSFYHLEKYkdrVSWVPNKHY 198 starlet sea ane...
Feature 1               #               # ###                           #                       #
AAU12251     218 SGIHGLMKLVLTKTlpsdlEKVIVLDTDITFATDIAELWvvfhkFKGQQVLGLVENQSDWYLGnlwKNHRPWPALGRGFN 297 zebrafish
O95461       217 SGIYGLMKLVLTKTlpanlERVIVLDTDITFATDIAELWavfhkFKGQQVLGLVENQSDWYLGnlwKNHRPWPALGRGYN 296 human
Q21389       122 SKYYGLSKLLIPEIigndiGKIMFMDVDIIFQTNIFDLWkqfrnFNNSQVFGMVENLSDWYLNk-dGKKSVWPALGRGFN 200 nematode
NP_001004383 217 SGIYGLMKLVLTKTlpsnlERVIVLDTDITFATDIAELWavfhkFKGQQVLGLVENQSDWYLGnlwKNHRPWPALGRGYN 296 chicken
AAH60410     186 SGIYGLLKLTLTKAlpsdlSKVIVLDTDITFATDIAELWaifkkFTGEQVLGLVENQSDWYLGnlwKNHKPWPALGRGFN 265 African clawed ...
XP_001176040  84 SGLYGLMKLLLIEIlpsnmEKTIVLDTDITFASDIAELWglfqrMTSKQTLALVENQSDWYLGtlwKKHKPWPALGRGFN 163 purple urchin
XP_624139    173 SGVYGLLKLILPDAmr--eDKVLVFDTDVTVLNDVSLLWhifekFSSDQALGLTENQSHWYIKalsYGQRPWPALGRGFN 250 honey bee
XP_001640724 199 SGVFGLMKLVLTEAlpevlDKVIVLDTDVIFASDVAELWklldqLTKKKAIGLVENQSDWYLGklwKNHKPWPALGRGFN 278 starlet sea ane...
Feature 1        ##                                   ##                        ##               
AAU12251     298 TGVILLLLDrLRKLKWEQMWRLTAERElmsmlsTSLADQDIFNAVIKQNPflVHQLPCYWNVQLSDHTRSekcykdvsdL 377 zebrafish
O95461       297 TGVILLLLDkLRKMKWEQMWRLTAERElmgmlsTSLADQDIFNAVIKQNPflVYQLPCFWNVQLSDHTRSeqcyrdvsdL 376 human
Q21389       201 TGIIMFDLDkLRKNGWASKWRVVANKYlrihgkTAMSDQDIFNAYIHDYPteIIQIPCAYNYQLGALTKSkelc--petP 278 nematode
NP_001004383 297 TGVILLLLDkLRKMKWEQMWRLTAERElmsmlsTSLADQDIFNAVIKQNPflVYQLPCFWNVQLSDHTRSeqcyrdvsdL 376 chicken
AAH60410     266 TGVILLLLDkLRLIGWEEMWRLTAERElmnmlsTSLADQDIFNAVIKSSPtlVYQLPCYWNVQLSDHTRSeqcyseladL 345 African clawed ...
XP_001176040 164 TGVILLDLQkLRDLKWMQMWLLTAEKElmsqfsTALADQDIINAVIKQHPylVYTLPCAWNVQLSDNTRSelcykevtdL 243 purple urchin
XP_624139    251 TGVMLMHLQqLRKRKFTSLWEAVTKRVlahipeTSLADQDIINAVIKERPfiVYKIKCTWNIQLSDHTISdicyrdtsqI 330 honey bee
XP_001640724 279 TGVILLDLNkLRRMNWKDKWRLTAEKElmtmlsTALADQDIFNAVIKNDPhlLHKLPCTWNVQLGDNTRSeqcytevneL 358 starlet sea ane...
Feature 1           # ##  #                               
AAU12251     378 KVIHWNSpKKLRVKNkhVEFFRNLYLTFLEYDGNLLRRELF 418 zebrafish
O95461       377 KVIHWNSpKKLRVKNkhVEFFRNLYLTFLEYDGNLLRRELF 417 human
Q21389       279 LALHFNSqNKTVGKN--YAFFDKIRKAFDEMDGSDLKRRRR 317 nematode
NP_001004383 377 KVIHWNSpKKLRVKNkhVEFFRNLYLTFLEYDGNLLRRELF 417 chicken
AAH60410     346 KVIHWNSpHKLRVKNkhVELFRTLYLTFLEYDGSLLRRELI 386 African clawed frog
XP_001176040 244 KAIHWNSpKKLRVKNkhVEFFRNLYLTFLEYDGNLLRRELF 284 purple urchin
XP_624139    331 SIIHWNSpRKQDVYNkhINEFRKLHRVFLEMDGNLLRRRLF 371 honey bee
XP_001640724 359 KVIHWNSpKKLLVKNkhGDFFRNLYLTFLEYDGNLLRRALF 399 starlet sea anemone

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