Calpain p94

Calpain p94 is a member of the calpain family, a group of calcium-dependent cysteine proteases. It is also known as CAPN3 and is specifically expressed in skeletal muscle.

Function

Calpain p94 is involved in the remodeling and maintenance of sarcomeres, the basic unit of muscle. It is activated by calcium and can cleave various substrates, including titin, a large protein that functions as a molecular spring which is responsible for the passive elasticity of muscle.

Clinical significance

Mutations in the CAPN3 gene are associated with limb-girdle muscular dystrophy type 2A (LGMD2A), a form of muscular dystrophy that affects the muscles of the hips and shoulders. This condition is characterized by progressive muscle weakness and wasting, and is one of the most common forms of limb-girdle muscular dystrophy.

Research

Research into calpain p94 is ongoing, with studies focusing on its role in muscle function and its potential as a therapeutic target for muscular dystrophies and other muscle diseases.

See also

• Calpain
• Limb-girdle muscular dystrophy
• Sarcomere
• Titin