Conserved Protein Domain Family
DAG1

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pfam05454: DAG1 
Dystroglycan (Dystrophin-associated glycoprotein 1)
Dystroglycan is one of the dystrophin-associated glycoproteins, which is encoded by a 5.5 kb transcript in human. The protein product is cleaved into two non-covalently associated subunits, [alpha] (N-terminal) and [beta] (C-terminal). In skeletal muscle the dystroglycan complex works as a transmembrane linkage between the extracellular matrix and the cytoskeleton. [alpha]-dystroglycan is extracellular and binds to merosin ([alpha]-2 laminin) in the basement membrane, while [beta]-dystroglycan is a transmembrane protein and binds to dystrophin, which is a large rod-like cytoskeletal protein, absent in Duchenne muscular dystrophy patients. Dystrophin binds to intracellular actin cables. In this way, the dystroglycan complex, which links the extracellular matrix to the intracellular actin cables, is thought to provide structural integrity in muscle tissues. The dystroglycan complex is also known to serve as an agrin receptor in muscle, where it may regulate agrin-induced acetylcholine receptor clustering at the neuromuscular junction. There is also evidence which suggests the function of dystroglycan as a part of the signal transduction pathway because it is shown that Grb2, a mediator of the Ras-related signal pathway, can interact with the cytoplasmic domain of dystroglycan. In general, aberrant expression of dystrophin-associated protein complex underlies the pathogenesis of Duchenne muscular dystrophy, Becker muscular dystrophy and severe childhood autosomal recessive muscular dystrophy. Interestingly, no genetic disease has been described for either [alpha]- or [beta]-dystroglycan. Dystroglycan is widely distributed in non-muscle tissues as well as in muscle tissues. During epithelial morphogenesis of kidney, the dystroglycan complex is shown to act as a receptor for the basement membrane. Dystroglycan expression in mouse brain and neural retina has also been reported. However, the physiological role of dystroglycan in non-muscle tissues has remained unclear.
Statistics
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PSSM-Id: 398879
Aligned: 4 rows
Threshold Bit Score: 425.565
Threshold Setting Gi: 688609841
Created: 20-May-2020
Updated: 7-Aug-2020
Structure
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Aligned Rows:
PubMed ReferencesClick to see Conserved Features Help

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
Q14118       606 ARFKAKFVGDPALVLNDIHKKiaLVKKLAFAFGDRNCSTITLQNIT----RGSIVVEWTNNTL--PLEPCPKEQIAGLSR 679  human
Q8STB9       724 FFFKAYLSIKHERFNADLQRK--FVERVAKLNGDPTTGQIQIRSITthhdSDGTIVNFYNTTLyrKHNSCREKEVAMTRS 801  fruit fly
XP_009294762 580 VLFTAVFDGDARTVTNDIHKKilLVKKLSQSFGDRNSSTITLKSIT----KGSIIVEWTNNSL--QQSPCPKDQIQQLSK 653  zebrafish
NP_001016518 594 VKFHAKFHGDHNAVINDINKKilLVKKLAFAFGDRNSSSITLHNIT----KGSVVVDWTNNTF--PVEPCPVEQVEGVGK 667  tropical clawe...
Q14118       680 RIAEDDGKPRPAFSNALEPDFKATSITVTGSGSCRH--LQFIPVVPPRRVPSEapptevPDRDPEKSSEDDVYLHTVIPA 757  human
Q8STB9       802 VYLNSDLSLREAAKRALGPELNLTNFSVVPFSICHH--TENIDTNQLDYIPSR------PEEPTHKSSFGEDYMITFVWP 873  fruit fly
XP_009294762 654 KISDPEGKPSSIFKFTMEPDFRPSNITVRGTASCRN--YMFVPL---GEIPDPtpspgtPAVGAGRQSTDDVYLHTVIPA 728  zebrafish
NP_001016518 668 KIYDERGSPRQHFVNAVEPEFKLLNISLSFSGSCKHkkFRYIPMRAEEPIPTAvaptvaVDRNLEKRSEDDVYLHTVIPA 747  tropical clawe...
Q14118       758 VVVAAILLIAGIIAMICYR--KKRKGKLTL---EDQATFIKKGVPIIFADELDDsKPPPSSSMPLILQEEKAPLPPPEYP 832  human
Q8STB9       874 IVIIVAMLVAASIIACCLHwcRQRSGKMELgdeEERKSFRAKGIPVIFQDEYEE-KPEIGNKSPVILKDEKPPLLPPSYN 952  fruit fly
XP_009294762 729 VVVAAILLIAGIIAMICYR--KKRKGKLTI---EDQATFIKKGVPIIFADELDDsKPPPSSSMPLILQEEKPPLPPPEYP 803  zebrafish
NP_001016518 748 VVVAAILLIAGIIAMICYR--KKRKGKLTI---EDQATFIKKGVPIIFADELDDsKPPPSSSMPLILKEEKAPLPPPEYP 822  tropical clawe...
Q14118       833 NQSVpettplnqdtmgeytplrDEDPNAPPYQPPPPFTAPMEGKGSRPKNMTPYRSPPPYVPP 895  human
Q8STB9       953 TSNM------------------NGDNDVDDYVPPPSVVVGGREVRGKSPATPSYRKPPPYVSP 997  fruit fly
XP_009294762 804 NMATpettplnqdllgeytplhDEDPNAPPYQPPPPFSTPMEGKGSRPKNMTPYRSPPPYVPP 866  zebrafish
NP_001016518 823 NQNVpetiplnqdslgeytplrDEDPNAPPYQPPPPFTAPMEGKGSRPKNMTPYRSPPPYVPP 885  tropical clawed frog
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