Harman Patil (Editor)

Sarcoglycan

Updated on
Edit
Like
Comment
Share on FacebookTweet on TwitterShare on LinkedInShare on Reddit
Symbol
  
Sarcoglycan_1

InterPro
  
IPR006875

PDB
  
RCSB PDB; PDBe; PDBj

Pfam
  
PF04790

Pfam
  
structures

PDBsum
  
structure summary

The sarcoglycans are a family of transmembrane proteins (α, β, γ, δ or ε) involved in the protein complex responsible for connecting the muscle fibre cytoskeleton to the extracellular matrix, preventing damage to the muscle fibre sarcolemma through shearing forces.

The dystrophin glycoprotein complex (DGC) is a membrane-spanning complex that links the interior cytoskeleton to the extracellular matrix in muscle. The sarcoglycan complex is a subcomplex within the DGC and is composed of several muscle-specific, transmembrane proteins (alpha-, beta-, gamma-, delta- and zeta-sarcoglycan). The sarcoglycans are asparagine-linked glycosylated proteins with single transmembrane domains.

The disorders caused by the mutations of the sarcoglycans are called sarcoglycanopathies. Mutations in the α, β, γ or δ genes (not ε) encoding these proteins can lead to the associated limb-girdle muscular dystrophy.

Genes

  • SGCA
  • SGCB
  • SGCD
  • SGCE
  • SGCG
  • SGCZ
  • References

    Sarcoglycan Wikipedia