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: 17-Jan-2013
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:
                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
Feature 1             ###   ##                                                                   
gi 51872295  139 IHIAIVCAg-yNASRDVVTLVKSVLFHRrnPLHFHIITDSIARKILADLFHTWmvpavhvNFYDADELkseVSWIPNKHY 217
gi 22001684  138 IHVAIVCAg-yNASRDVVTLVKSVLFHRrnPLHFHLIADSIAEQILATLFQTWmvpavrvDFYNADELkseVSWIPNKHY 216
gi 92058691   42 IRLAYIIGgnfMTRLMFMQHFKSVLKYSdhFFRLHLITDENHRSDIHELMTSWnisncewFFHNLTEFekrVAWIPNSHY 121
gi 52138661  138 IHVAIVCAg-yNASRDVVTLVKSVLFHRrnPLHFHLIADAIAKQILATLFQTWmvpavriDFYDADELkseVSWIPNKHY 216
gi 38014447  107 IHVAIVCAg-hNSSRDVVTLVKSILFHRrnPLHLHLITDDVALRVLGNLFNTWmvpslqiSFYNASELkpdVAWIPNKHY 185
gi 115908500   5 IHVSIVCAg-hNASRDVIILIKSILFYRknPLHFHFISDEMGQLILSTLFKTWdvpgvkvSFYLAENYkshVEWIPNKHY 83
gi 66511271   94 VHIAMVCAg-yNSTFALVTVVKSVLFYRtkPLHFHLLVDEIAKRTLTTVFQTWdlphvnlTYYKAEKWvpkVSWIPNKHY 172
gi 156405409 120 IHIAVVCAg-hSASRQAVTLIKSLLFYRhnPLHFHFVSDSVAELILGTLFRTWgvpavqtSFYHLEKYkdrVSWVPNKHY 198
                         90       100       110       120       130       140       150       160
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
Feature 1               #               # ###                           #                       #
gi 51872295  218 SGIHGLMKLVLTKTlpsdlEKVIVLDTDITFATDIAELWvvfhkFKGQQVLGLVENQSDWYLGnlwKNHRPWPALGRGFN 297
gi 22001684  217 SGIYGLMKLVLTKTlpanlERVIVLDTDITFATDIAELWavfhkFKGQQVLGLVENQSDWYLGnlwKNHRPWPALGRGYN 296
gi 92058691  122 SKYYGLSKLLIPEIigndiGKIMFMDVDIIFQTNIFDLWkqfrnFNNSQVFGMVENLSDWYLNk-dGKKSVWPALGRGFN 200
gi 52138661  217 SGIYGLMKLVLTKTlpsnlERVIVLDTDITFATDIAELWavfhkFKGQQVLGLVENQSDWYLGnlwKNHRPWPALGRGYN 296
gi 38014447  186 SGIYGLLKLTLTKAlpsdlSKVIVLDTDITFATDIAELWaifkkFTGEQVLGLVENQSDWYLGnlwKNHKPWPALGRGFN 265
gi 115908500  84 SGLYGLMKLLLIEIlpsnmEKTIVLDTDITFASDIAELWglfqrMTSKQTLALVENQSDWYLGtlwKKHKPWPALGRGFN 163
gi 66511271  173 SGVYGLLKLILPDAmr--eDKVLVFDTDVTVLNDVSLLWhifekFSSDQALGLTENQSHWYIKalsYGQRPWPALGRGFN 250
gi 156405409 199 SGVFGLMKLVLTEAlpevlDKVIVLDTDVIFASDVAELWklldqLTKKKAIGLVENQSDWYLGklwKNHKPWPALGRGFN 278
                        170       180       190       200       210       220       230       240
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
Feature 1        ##                                   ##                        ##               
gi 51872295  298 TGVILLLLDrLRKLKWEQMWRLTAERElmsmlsTSLADQDIFNAVIKQNPflVHQLPCYWNVQLSDHTRSekcykdvsdL 377
gi 22001684  297 TGVILLLLDkLRKMKWEQMWRLTAERElmgmlsTSLADQDIFNAVIKQNPflVYQLPCFWNVQLSDHTRSeqcyrdvsdL 376
gi 92058691  201 TGIIMFDLDkLRKNGWASKWRVVANKYlrihgkTAMSDQDIFNAYIHDYPteIIQIPCAYNYQLGALTKSkelc--petP 278
gi 52138661  297 TGVILLLLDkLRKMKWEQMWRLTAERElmsmlsTSLADQDIFNAVIKQNPflVYQLPCFWNVQLSDHTRSeqcyrdvsdL 376
gi 38014447  266 TGVILLLLDkLRLIGWEEMWRLTAERElmnmlsTSLADQDIFNAVIKSSPtlVYQLPCYWNVQLSDHTRSeqcyseladL 345
gi 115908500 164 TGVILLDLQkLRDLKWMQMWLLTAEKElmsqfsTALADQDIINAVIKQHPylVYTLPCAWNVQLSDNTRSelcykevtdL 243
gi 66511271  251 TGVMLMHLQqLRKRKFTSLWEAVTKRVlahipeTSLADQDIINAVIKERPfiVYKIKCTWNIQLSDHTISdicyrdtsqI 330
gi 156405409 279 TGVILLDLNkLRRMNWKDKWRLTAEKElmtmlsTALADQDIFNAVIKNDPhlLHKLPCTWNVQLGDNTRSeqcytevneL 358
                        250       260       270       280
                 ....*....|....*....|....*....|....*....|.
Feature 1           # ##  #                               
gi 51872295  378 KVIHWNSpKKLRVKNkhVEFFRNLYLTFLEYDGNLLRRELF 418
gi 22001684  377 KVIHWNSpKKLRVKNkhVEFFRNLYLTFLEYDGNLLRRELF 417
gi 92058691  279 LALHFNSqNKTVGKN--YAFFDKIRKAFDEMDGSDLKRRRR 317
gi 52138661  377 KVIHWNSpKKLRVKNkhVEFFRNLYLTFLEYDGNLLRRELF 417
gi 38014447  346 KVIHWNSpHKLRVKNkhVELFRTLYLTFLEYDGSLLRRELI 386
gi 115908500 244 KAIHWNSpKKLRVKNkhVEFFRNLYLTFLEYDGNLLRRELF 284
gi 66511271  331 SIIHWNSpRKQDVYNkhINEFRKLHRVFLEMDGNLLRRRLF 371
gi 156405409 359 KVIHWNSpKKLLVKNkhGDFFRNLYLTFLEYDGNLLRRALF 399

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