Calpastatin
Domain
Each of the four flexible inhibitory domains can inhibit one calcium-bound calpain molecule by occupying both sides of the active site.
Function
Specific inhibition of calpain (calcium-dependent cysteine protease). Plays a key role in postmortem tenderization of meat and have been proposed to be involved in muscle protein degradation in living tissue.
Involvement in disease
Peeling skin with leukonychia, acral punctate keratoses, cheilitis, and knuckle pads
PLACK
An autosomal recessive disease characterized by generalized, continuous shedding of the outer layers of the epidermis, leukonychia, acral punctate keratosis, cheilitis, knuckle pads with multiple hyperkeratotic micropapules involving the interphalangeal joints, and palmoplantar keratoderma.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
The N-terminus is blocked.
Sequence Similarities
Belongs to the protease inhibitor I27 (calpastatin) family.
Alternative names
Calpastatin, Calpain inhibitor, Sperm BS-17 component, CAST