Recombinant Human Utrophin protein

Supplementary info

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

Activity summary

Utrophin also known as dystrophin-related protein plays a mechanical role in muscle tissue by connecting the cytoskeleton to the extracellular matrix. It has a molecular mass of about 395 kDa. This protein localizes mainly at the neuromuscular junctions and myotendinous junctions. Utrophin shows expression in various tissues but is more common in fetal and regenerating muscle fibers suggesting its function is important during muscle development and repair.

Biological function summary

Utrophin maintains muscle integrity and stability by forming part of the dystrophin-associated glycoprotein complex. This complex acts as a scaffold needed for structural support in muscle cells aiding in proper muscle function and repair. Utrophin substitutes dystrophin especially when dystrophin is absent or defective in muscles making it an important player in compensatory mechanisms.

Pathways

Utrophin actively participates in muscle signaling and structural integrity processes. It is important within the Jak-STAT signaling pathway and the pathways involved in muscle dystrophy. Through these pathways utrophin interacts with proteins like dystroglycans and sarcoglycans which help support its role in maintaining the structural framework of muscle tissues.

Associated diseases and disorders

The absence or abnormal expression of utrophin is linked to muscular dystrophies especially Duchenne Muscular Dystrophy (DMD). Utrophin is being explored as a potential therapeutic target to compensate for the lack of dystrophin in this context. Enhanced expression of utrophin may alleviate muscle degeneration seen in DMD by interacting with proteins normally associated with dystrophin in healthy muscle like syntrophins and sarcospan.