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Protein Domain : IPR008465

Name  Dystroglycan Short Name  DAG1
Type  Family Description  Dystroglycan is one of the dystrophin-associated glycoproteins, which is encoded by a 5.5 kb transcript in Homo sapiens. 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 inmuscle tissues. During epithelial morphogenesis of kidney, the dystroglycan complex is shown to act as a receptor for the basement membrane. Dystroglycan expression in Mus musculusbrain and neural retina has also been reported. However, the physiological role of dystroglycan in non-muscle tissues has remained unclear [].

Publication Counts Displayer

0 Child Features

2 Contains

DB identifier Name Short Name Type
IPR013783 Immunoglobulin-like fold Ig-like_fold Domain
IPR006644 Dystroglycan-type cadherin-like Cadg Domain

1 Cross References

Identifier
Source . Name

Subject . Primary Identifier
PF05454 PFAM IPR008465

3 Data Sets

Name URL
TrEMBL data set http://www.ebi.ac.uk/trembl/
InterPro data set  
InterPro GO Annotation data set  

0 Found In

2 GO Annotation


Subject . Secondary Identifier

Subject . Name

Subject . Symbol

Ontology Term . Identifier

Ontology Term . Name
Dystroglycan   GO:0007016 cytoskeletal anchoring at plasma membrane
Dystroglycan   GO:0016010 dystrophin-associated glycoprotein complex

2 Ontology Annotations


Subject . Secondary Identifier

Subject . Name

Subject . Symbol

Ontology Term . Identifier

Ontology Term . Name
Dystroglycan   GO:0007016 cytoskeletal anchoring at plasma membrane
Dystroglycan   GO:0016010 dystrophin-associated glycoprotein complex

0 Parent Features

23 Proteins

DB identifier Primary Accession
Organism . Name
FBpp0086395 A1ZA89 Drosophila melanogaster
FBpp0086394 Q8STB9 Drosophila melanogaster
FBpp0086396 Q8WR08 Drosophila melanogaster
FBpp0307072 N6VFR5 Drosophila pseudoobscura
FBpp0307074 N6W556 Drosophila pseudoobscura
FBpp0307073 N6V903 Drosophila pseudoobscura
FBpp0275979 Q28XG7 Drosophila pseudoobscura
FBpp0169627 B4KQB4 Drosophila mojavensis
FBpp0234498 B4LL53 Drosophila virilis
FBpp0259111 B4P6R8 Drosophila yakuba
A8XR81_CAEBR A8XR81 Caenorhabditis briggsae
A8XNU3_CAEBR A8XNU3 Caenorhabditis briggsae
H2WCN5_CAEJA H2WCN5 Caenorhabditis japonica
G0MH60_CAEBE G0MH60 Caenorhabditis brenneri
G0MHL4_CAEBE G0MHL4 Caenorhabditis brenneri
G0MH69_CAEBE G0MH69 Caenorhabditis brenneri
Q19173_CAEEL Q19173 Caenorhabditis elegans
O61756_CAEEL O61756 Caenorhabditis elegans
Q22629_CAEEL Q22629 Caenorhabditis elegans
Q8WR09_DROME Q8WR09 Drosophila melanogaster
Q8T0G6_DROME Q8T0G6 Drosophila melanogaster
E3MAI4_CAERE E3MAI4 Caenorhabditis remanei
E3M2F1_CAERE E3M2F1 Caenorhabditis remanei