1G13


Conserved Protein Domain Family
GM2-AP

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cd00258: GM2-AP 
Click on image for an interactive view with Cn3D
GM2 activator protein (GM2-AP) is a non-enzymatic lysosomal protein that acts as cofactor in the sequential degradation of gangliosides. GM2A is an essential cofactor for beta-hexosaminidase A (Hex A) in the enzymatic hydrolysis of GM2 ganglioside to GM3. Mutation of the gene results in the AB variant of Tay-Sachs disease. GM2-AP and similar proteins belong to the ML domain family.
Statistics
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PSSM-Id: 238161
View PSSM: cd00258
Aligned: 3 rows
Threshold Bit Score: 286.006
Threshold Setting Gi: 14278639
Created: 4-Sep-2001
Updated: 2-Oct-2020
Structure
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Program:
Drawing:
Aligned Rows:
 
putative lipid
Conserved site includes 35 residues -Click on image for an interactive view with Cn3D
Feature 1:putative lipid binding cavity [chemical binding site]
Evidence:
  • Comment:hydrophobic cavity of conserved residues

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
Feature 1       # #         # #  # #    #     # # #             # # #                #    #  #
1G13_A      1 SSFSWDNCDEGKDPAVIRSLTLEPDPIIVPGNVTLSVXGSTSVPLSSPLKVDLVLEKEVAGLWIKIPCTDYIGSCTFEHF 80  human
AAH74424   32 NGFSWSNCDGESLPGKIKSLSVNPNPINIPGDLTVSTVLETKVPLTSPVKVIITAEKELLGEWMKVPCLDNIGSCTYDNA 111 African clawed frog
CAG04534   35 QGFDWKNCGRPDVPAVLQSLTVSPDPIAIPGDLTASASGSTSVELSSPLSLNLTLEKEVAGLWVKIPCVEQLGSCHYRDA 114 Tetraodon nigrovir...
Feature 1          ###      # #           #      #    #  # #          # # # #      #  # # # # 
1G13_A     81 CDVLDXLIPTGEPCPEPLRTYGLPCHCPFKEGTYSLPKSEFVVPDLELPSWLTTGNYRIESVLSSSGKRLGCIKIAASLK 160 human
AAH74424  112 CELIDTIFPPGQQCPEPLRTYGLPCHCPFKEGVYSLPDTTLTLPNVDLPSWLANGNYRITGILMADNKEIGCGKFTFSLE 191 African clawed frog
CAG04534  115 CDLLSRLIPPGQDCPEPLHTYGLPCRCPFKAGSYALPQSQFYLPYVDLPYWLTNGNYRVQGLLGGEGQELGCLKLSLSIH 194 Tetraodon nigrovir...
Feature 1       
1G13_A    161 GI 162 human
AAH74424  192 SS 193 African clawed frog
CAG04534  195 SE 196 Tetraodon nigroviridis

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