Conserved Protein Domain Family
CBM20_laforin

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cd05806: CBM20_laforin 
Laforin protein tyrosine phosphatase, N-terminal CBM20 (carbohydrate-binding module, family 20) domain. Laforin, encoded by the EPM2A gene, is a dual-specificity phosphatase that dephosphorylates complex carbohydrates. Mutations in the gene encoding laforin result in Lafora disease, a fatal autosomal recessive neurodegenerative disorder characterized by the presence of intracellular deposits of insoluble, abnormally branched, glycogen-like polymers, known as Lafora bodies, in neurons, muscle, liver, and other tissues. The molecular basis for the formation of these Lafora bodies is unknown. Laforin is one of the only phosphatases that contains a carbohydrate-binding module. The CBM20 domain is found in a large number of starch degrading enzymes including alpha-amylase, beta-amylase, glucoamylase, and CGTase (cyclodextrin glucanotransferase). CBM20 is also present in proteins that have a regulatory role in starch metabolism in plants (e.g. alpha-amylase) or glycogen metabolism in mammals (e.g. laforin). CBM20 folds as an antiparallel beta-barrel structure with two starch binding sites. These two sites are thought to differ functionally with site 1 acting as the initial starch recognition site and site 2 involved in the specific recognition of appropriate regions of starch.
Statistics
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PSSM-Id: 99881
View PSSM: cd05806
Aligned: 5 rows
Threshold Bit Score: 164.997
Threshold Setting Gi: 156357458
Created: 10-Sep-2007
Updated: 17-Jan-2013
Structure
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Aligned Rows:
 
starch-bindingstarch-binding
Feature 1:starch-binding site 1 [chemical binding site]
Evidence:
  • Comment:Based on the binding of substrates to the starch binding site 1 of other family members.
  • Comment:Site 1 is small relative to site 2 and exhibits minimal structural changes upon ligand binding. It acts as an initial starch recognition site.
  • Citation:PMID 9195884
  • Citation:PMID 8672460

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
Feature 1                                       #                                                
gi 122134326   1 MRFRFGVVvppaGAGAAPELLVVGSRPELGRWEp---------RGAVRLRPagsaagggaralqEPGLWLGEVELAPgea 71
gi 148228643   1 MLFRFGVVi--tLNHSDNELLVLGSRKEMGSWDp---------QYAVPMKPtrcp-----lsttEPSFWVGEVLLEEpy- 63
gi 47224605    1 MLFRFGVIl--tPDCPDVDVFVLGSRVEMGEWDp---------GRAVPMRAsrkr-----lsphEPCLWTGDVELAEpf- 63
gi 156357458   3 ITVKFSVVt---FIDRETTVSVLGSVPELGSWNqknrieltqsDSEFRLGPg-----------lEPSFWSKDVEMSFngk 68
gi 71894761    1 MLFRFGVVlparIAEGGGALLVAGSRPELGEWDp---------QRAVPMQParpaa---alaaqEPVLWLGEVLLSDed- 67
                         90       100       110       120       130       140
                 ....*....|....*....|....*....|....*....|....*....|....*....|....
Feature 1                       #           ##   #                               
gi 122134326  72 ardgaeparvDTFWYKFLKREpggaLSWEGNGPHHDRCCTY------NENNLVDGVYCLPIGHW 129
gi 148228643  64 ---------tDTFWFKFIKKEag-aLIWEGNGPHHDRCCVY------DDSNIVDGVYCHPVGHW 111
gi 47224605   64 ---------qDMLWFKFLKRVad-tTIWEGNGPSHDRCCVY------DTRNMVDGVYCHPVGHW 111
gi 156357458  69 --------seLRFEYKFVSHKng-nFIWEGEGPCHNRKEVLtlndktASELLVNGVYILPVGIW 123
gi 71894761   68 --------taSPFWYKFLRREgg-qLLWEGNGPHHDRSCVY------NQSNIVDGVYCLPIAHW 116

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