Contents

Search

dystrophin associated protein complex (DPC)

Function: - stabilize sarcolemma, protect muscle fibers from long-term contraction induced damage and necrosis - possible role in cellular communication: acting as transmembrane signaling complex [3] - dystroglycan ligands include: a) laminins b) agrin c) perlecan d) neurexin e) nidogen f) biglycan Structure: components: - dystroglycan complex: alpha & beta dystroglycans - sarcoglycan complex: - alpha, beta, gamma & delta, zeta sarcoglycans - sarcospan - syntrophin - alpha-dystrobrevin - nNOS - SAPK3 - SAST (syntrophin associated serine/threonine kinase) - skeletal muscle voltage gated sodium channels - intermediate filament proteins a) syncoilin b) desmuslin c) synemin - aquaporin 4 - in non-muscle tissue, alpha-neurexin & erbB4 are also associated with components of the DPC.

Related

dystrophin-associated glycoprotein complex (GO:0016010)

General

molecular complex

Properties

SUBUNITS: dystroglycan 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} sarcoglycan MOTIF: glycosylation site membrane region sarcospan MOTIF: cytoplasmic domain {1-53} transmembrane domain {54-74} exoplasmic loop {75-86} transmembrane domain {87-107} cytoplasmic loop {108-122} transmembrane domain {123-143} exoplasmic loop {144-193} transmembrane domain {194-214} cytoplasmic domain {215-243} syntrophin MOTIF: DHR domain ALPHA-DYSTROBREVIN NO synthase-1 CONFIGURATION: dimer MOTIF: NOSIP interaction {1-205} MOTIF: PDZ domain NAME: PDZ domain SITE: 17-99 PIN (nNOS-inhibiting protein) binding {163-245} Iron [Fe]-binding site SITE: 420-420 binding site SITE: 730-750 FOR-BINDING-OF: calmodulin binding site SITE: 755-774 FOR-BINDING-OF: 5,6,7-8-tetrahydrobiopterin Flavodoxin-like {760-940} cofactor-binding site [1032-1043] COFACTOR-BOUND: flavin adenine dinucleotide cofactor-binding site [1175-1185] COFACTOR-BOUND: flavin adenine dinucleotide cofactor-binding site [1250-1268] COFACTOR-BOUND: NADP cofactor-binding site [1348-1363] COFACTOR-BOUND: NADP mitogen-activated protein kinase 12 COMPARTMENT: mitochondria cytoplasm STATE: active state MOTIF: kinase domain SITE: 27-311 MOTIF: ATP-binding site NAME: ATP-binding site SITE: 33-41 Tyr phosphorylation site {Y27} ATP-binding site NAME: ATP-binding site SITE: 56-56 aspartate residue {D153} TXY {183-185} Thr phosphorylation site {T183} Tyr phosphorylation site {Y185} syntrophin associated serine/threonine kinase STATE: active state MOTIF: S/T phosphorylation site kinase domain MOTIF: ATP-binding site NAME: ATP-binding site

References

  1. Blake D & Martin-Rendon E Intermediate filaments and the function of the dystrophin- protein complex. Trends Cardiovasc Med 12(5):224-8, 2002 PMID: 12161077
  2. Michele DE & Campbell KP Dystrophin-glycoprotein complex: post-translational processing and dystroglycan function. J Biol Chem 278(18):15457-60, 2003 PMID: 12556455
  3. Muntoni F et al Dystrophin and mutations: one gene, several proteins, multiple phenotypes. Lancet Neurology 2:731-40, 2003 PMID: 14636778

Components

dystroglycan; dystrophin-associated glycoprotein 1; contains: alpha-dystroglycan (alpha-DG), beta-dystroglycan (beta-DG); DAG1 mitogen-activated protein kinase 12; extracellular signal-regulated kinase 6; ERK-6; ERK5; stress-activated protein kinase 3; mitogen-activated protein kinase p38 gamma; MAP kinase p38 gamma (MAPK12, ERK6, SAPK3) NO synthase-1 (calmodulin NO synthase, NADPH diaphorase, neuronal NO synthase, nNOS) sarcoglycan sarcospan; K-ras oncogene-associated protein; Kirsten-ras-associated protein (SSPN, KRAG) syntrophin (dystrophin-associated protein) syntrophin associated serine/threonine kinase (SAST)