SGCG_HUMAN » Gamma-sarcoglycan

SGCG_HUMAN » Gamma-sarcoglycan
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
SGCG_HUMAN » Gamma-sarcoglycan » Gamma-SG; 35 kDa dystrophin-associated glycoprotein;35DAG;
Hydrophobic Thickness 38.4 ± 1.8 Å
Tilt Angle 1 ± 3°
ΔGtransfer -49.0 kcal/mol
ΔGfold -23.4 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology In
TM Segments 28-60 (28-64)
Pathways

Arrhythmogenic right ventricular cardiomyopathy (KEGG)

Dilated cardiomyopathy (KEGG)

Hypertrophic cardiomyopathy (KEGG)

Viral myocarditis (KEGG)

PDB none
OPM none
Complexes none
Interactions

A4, Complex: A4:SGCG, PubMed

DYSF, Complex: SGCG:DYSF, PubMed

SGCB, Complex: SGCG:SGCB, PubMed

SGCD, Complex: SGCG:SGCD, PubMed

Domains

AA: 25-279, Sarcoglycan complex subunit protein

UniProt annotation for SGCG_HUMAN » Gamma-sarcoglycan
FUNCTION: Component of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix.

SUBUNIT: Interacts with the syntrophin SNTA1. Cross-link to form 2 major subcomplexes: one consisting of SGCB, SGCD and SGCG and the other consisting of SGCB and SGCD. The association between SGCB and SGCG is particularly strong while SGCA is loosely associated with the other sarcoglycans (By similarity). Interacts with FLNC.

TISSUE SPECIFICITY: Expressed in skeletal and heart muscle.

DISEASE: Limb-girdle muscular dystrophy 2C (LGMD2C) OMIM: An autosomal recessive degenerative myopathy characterized by rapidly progressive muscle wasting from early childhood with loss of independent ambulation around age 12 years, dystrophic pattern on muscle biopsy, absence of gamma-sarcoglycan and normal dystrophin immunostaining. mutations affecting the gene represented in this entry.

UniProt features for SGCG_HUMAN » Gamma-sarcoglycan
CHAIN 1 291 Gamma-sarcoglycan.
DISULFID 265 290 Potential.
DISULFID 267 283 Potential.
Amino Acid Sequence for SGCG_HUMAN » Gamma-sarcoglycan
MVREQYTTAT EGICIERPEN QYVYKIGIYG WRKRCLYLFV LLLLIILVVN LALTIWILKV MWFSPAGMGH LCVTKDGLRL EGESEFLFPL YAKEIHSRVD SSLLLQSTQN VTVNARNSEG EVTGRLKVGP KMVEVQNQQF QINSNDGKPL FTVDEKEVVV GTDKLRVTGP EGALFEHSVE TPLVRADPFQ DLRLESPTRS LSMDAPRGVH IQAHAGKIEA LSQMDILFHS SDGMLVLDAE TVCLPKLVQG TWGPSGSSQS LYEICVCPDG KLYLSVAGVS TTCQEHSHIC L