SGCB_HUMAN » Beta-sarcoglycan

SGCB_HUMAN » Beta-sarcoglycan
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
SGCB_HUMAN » Beta-sarcoglycan » Beta-SG; 43 kDa dystrophin-associated glycoprotein;43DAG; A3b;
Hydrophobic Thickness 41.2 ± 4.6 Å
Tilt Angle 2 ± 1°
ΔGtransfer -33.0 kcal/mol
ΔGfold -28.4 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology In
TM Segments 63-90 (63-92)
Pathways

Arrhythmogenic right ventricular cardiomyopathy (KEGG)

Dilated cardiomyopathy (KEGG)

Hypertrophic cardiomyopathy (KEGG)

Viral myocarditis (KEGG)

PDB none
OPM none
Complexes none
Interactions

SGCA, Complex: SGCE:SGCA:SGCB:SGCG:SGCD, PubMed

SGCD, Complex: SGCB:SGCD, PubMed

SGCG, Complex: SGCG:SGCB, PubMed

SGCZ, Complex: SGCB:SGCZ, PubMed

Domains

AA: 54-303, Sarcoglycan complex subunit protein

UniProt annotation for SGCB_HUMAN » Beta-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: 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).

TISSUE SPECIFICITY: Highest expression in heart and skeletal muscle. Low expression in brain, kidney, placenta, pancreas and lung. High expression in fetal brain. Also found in fetal lung, kidney and liver.

DISEASE: Limb-girdle muscular dystrophy 2E (LGMD2E) OMIM: An autosomal recessive degenerative myopathy characterized by pelvic and shoulder muscle wasting, onset usually in childhood and variable progression rate. disease is caused by mutations affecting the gene represented in this entry.

UniProt features for SGCB_HUMAN » Beta-sarcoglycan
CHAIN 1 318 Beta-sarcoglycan.
DISULFID 288 314 Potential.
DISULFID 290 307 Potential.
Amino Acid Sequence for SGCB_HUMAN » Beta-sarcoglycan
MAAAAAAAAE QQSSNGPVKK SMREKAVERR SVNKEHNSNF KAGYIPIDED RLHKTGLRGR KGNLAICVII LLFILAVINL IITLVIWAVI RIGPNGCDSM EFHESGLLRF KQVSDMGVIH PLYKSTVGGR RNENLVITGN NQPIVFQQGT TKLSVENNKT SITSDIGMQF FDPRTQNILF STDYETHEFH LPSGVKSLNV QKASTERITS NATSDLNIKV DGRAIVRGNE GVFIMGKTIE FHMGGNMELK AENSIILNGS VMVSTTRLPS SSSGDQLGSG DWVRYKLCMC ADGTLFKVQV TSQNMGCQIS DNPCGNTH