Anti-alpha Sarcoglycan antibody [EPR14773] - BSA and Azide free

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Alpha Sarcoglycan also known as Adhalin or 50DAG is a protein with a molecular mass of approximately 50 kDa. It is an important component in skeletal and cardiac muscle tissue. This protein is part of the dystrophin-glycoprotein complex (DGC) which connects the cytoskeleton of a muscle fiber to the extracellular matrix. Alpha Sarcoglycan is primarily expressed in striated muscle cells including those of skeletal and cardiac muscles but also at low levels in other tissues.

Biological function summary

Alpha Sarcoglycan contributes to the integrity and function of muscle fibers by stabilizing the DGC which includes sarcoglycans and other associated proteins. This stabilization provides mechanical support during muscle contraction and stretching. Alpha Sarcoglycan's role in the complex ensures proper signal transduction and protects muscle fibers from injury that may occur through mechanical stress. The protein's interaction with other sarcoglycans such as beta gamma and delta facilitates these structural and signaling functions.

Pathways

Alpha Sarcoglycan is involved in muscle development and maintenance pathways. It plays a role in the mechanotransduction signaling pathway which helps cells convert mechanical stimuli into chemical activity. The DGC including alpha Sarcoglycan works closely with dystrophin an important protein in the process. This relationship is essential for maintaining the structural integrity of muscle fibers and impacts pathways regulating muscle repair and regeneration.

Associated diseases and disorders

Mutations in the alpha Sarcoglycan gene are linked to limb-girdle muscular dystrophy type 2D (LGMD2D). This condition is characterized by progressive muscle weakness particularly affecting the shoulders and hips. The disruption of alpha Sarcoglycan affects its interaction with other sarcoglycan proteins leading to the disassembly of the DGC and causing increased muscle cell damage. Understanding the role of alpha Sarcoglycan can also provide insights into other disorders related to defects in the DGC.