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dystroglycan; dystrophin-associated glycoprotein 1; contains: alpha-dystroglycan (alpha-DG), beta-dystroglycan (beta-DG); DAG1
Function:
- part of the dystrophin-associated protein complex (DAPC) which may link cytoskeleton to the extracellular matrix
- mediates acetylcholine receptor aggregation
- alpha-dystroglycan functions as a laminin receptor
- Thr-379 is glycosylated by a trisaccharide with the structure 2-(N-acetylamido)-2-deoxygalactosyl-beta-1,3-2- (N-acetylamido)-2-deoxyglucosyl-beta-1,4-6-phosphomannose; this trisaccharide constitutes part of the ligand recognized by laminin & agrin
- interacts with SGCD (putative)
- interacts with AGR2 & AGR3
Structure:
- glycosylated form as large as 156-200 kD.
- contains 1 peptidase S72 domain
Compartment:
- alpha-dystroglycan: secreted, extracellular space
- beta-dystroglycan:
- cell membrane; single-pass type 1 membrane protein
- cytoplasm, cytoskeleton
Expression:
- expressed in a variety of fetal & adult tissues
Pathology:
- target for the entry of Mycobacterium leprae into peripheral nerve Schwann cells
- bound by cellular receptors of several arenaviruses
a) lymphocytic choriomeningitis virus
b) Lassa fever virus
c) Clade C New World arenaviruses
Specific
alpha dystroglycan (alpha DG)
beta dystroglycan (beta DG)
General
glycoprotein
membrane protein
Properties
SIZE: entity length = 895 aa
MW = 97 kD
COMPARTMENT: cytoplasm
cellular membrane
MOTIF: signal sequence {1-29}
N-glycosylation site {N141}
cysteine residue {C182}
MODIFICATION: cysteine residue {C264}
cysteine residue {C264}
MODIFICATION: cysteine residue {C182}
Thr glycosylation site {T379}
Thr glycosylation site {T381}
Thr glycosylation site {T388}
Ser glycosylation site {S485}
Peptidase S72 {500-733}
MOTIF: N-glycosylation site {N641}
N-glycosylation site {N649}
N-glycosylation site {N661}
cysteine residue {C669}
MODIFICATION: cysteine residue {C713}
cysteine residue {C713}
MODIFICATION: cysteine residue {C669}
transmembrane domain {750-775}
Database Correlations
OMIM 128239
UniProt Q14118
Pfam PF05454
Entrez Gene 1605
Kegg hsa:1605
References
- UniProt :accession Q14118
- Wikipedia; Dystroglycan entry
http://en.wikipedia.org/wiki/Dystroglycan
- Campanelli JT et al
A role for dystrophin-associated glycoproteins and utrophin
in agrin-induced AChR clustering.
PMID: 8205616
Cell 77:663-674 1994
- Gee SH et al
Dystroglycan-alpha, a dystrophin-associated glycoprotein,
is a functional agrin receptor.
Cell 77:675-686 1994
PMID: 8205617
- Entrez Gene :accession 1605
Component-of
dystrophin associated protein complex (DPC)