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alpha-1-syntrophin; 59 kD dystrophin-associated protein A1 acidic component 1; Pro-TGF-alpha cytoplasmic domain-interacting protein 1; TACIP1; syntrophin-1 (SNTA1 SNT1)
Function:
- adapter protein that binds to & probably organizes the subcellular localization of a variety of membrane proteins
- may link various receptors to the actin cytoskeleton & the extracellular matrix via the dystrophin glycoprotein complex
- plays a role in synapse formation & in organization of UTRN & acetylcholine receptors at the neuromuscular synapse
- binds to phosphatidylinositol 4,5-biphosphate (putative)
- phosphorylated by CaM-kinase 2; phosphorylation may inhibit interaction with DMD (putative)
- interacts with the other members of the syntrophin family SNTB1 & SNTB2; SGCG & SGCA of dystrophin glycoprotein complex; NOS1; GRB2; the Na+ channel proteins SCN4A & SCN5A; F-actin & calmodulin (putative)
- interacts with dystrophin protein DMD & related proteins DTNA & UTRN & with MAPK12, TGFA & GA
Structure:
- monomer & homodimer
- the 1st PH domain mediates oligomerization (Ca+2-dependent), & association with phosphatidylinositol 4,5-biphosphate (putative)
- the PDZ domain binds to the last 3 or 4 amino acids of ion channels & receptor proteins; association with dystrophin or related proteins probably leaves the PDZ domain available to recruit proteins to the membrane
- the SU domain binds calmodulin (Ca+2-dependent)
- belongs to the syntrophin family
- contains 1 PDZ domain (DHR domain)
- contains 2 PH domains
- contains 1 SU domain (syntrophin unique)
Compartment:
- cell membrane, sarcolemma
- peripheral membrane protein; cytoplasmic side (putative)
- cell junction (putative)
- cytoplasm, cytoskeleton
- in skeletal muscle, localizes at the cytoplasmic side of the sarcolemmal membrane & at neuromuscular junctions
Expression:
- high expression in skeletal muscle & heart
- low expression in brain, pancreas, liver, kidney & lung
- not detected in placenta
General
phosphoprotein
syntrophin (dystrophin-associated protein)
Properties
SIZE: entity length = 505 aa
MW = 54 kD
COMPARTMENT: cytoplasm
MOTIF: PH domain {6-269}
MOTIF: PDZ domain
NAME: PDZ domain
SITE: 87-170
Ser phosphorylation site {S189}
Ser phosphorylation site {S193}
PH domain {293-401}
SU {449-505}
MOTIF: binding site
SITE: 483-505
FOR-BINDING-OF: calmodulin
Database Correlations
OMIM 601017
MORBIDMAP 601017
UniProt Q13424
PFAM correlations
Entrez Gene 6640
Kegg hsa:6640
References
- UniProt :accession Q13424
- OMIM :accession 601017
- Entrez Gene :accession 6640