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SGCA

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.

Involvement in disease

Muscular dystrophy, limb-girdle, autosomal recessive 3

LGMDR3

An autosomal recessive degenerative myopathy characterized by progressive muscle wasting from early childhood with loss of independent ambulation by teenage years. Muscle biopsy shows necrosis, decreased immunostaining for alpha sarcoglycan, and adhalin deficiency.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence Similarities

Belongs to the sarcoglycan alpha/epsilon family.

Tissue Specificity

Most strongly expressed in skeletal muscle. Also expressed in cardiac muscle and, at much lower levels, in lung. In the fetus, most abundant in cardiac muscle and, at lower levels, in lung. Also detected in liver and kidney. Not expressed in brain.

Cellular localization

Alternative names

ADL, DAG2, SGCA, Alpha-sarcoglycan, Alpha-SG, 50 kDa dystrophin-associated glycoprotein, Adhalin, Dystroglycan-2, 50DAG

swissprot:Q16586 entrezGene:6442 omim:600119