... substrates.Through the absence of cleavage of these substrates, calpain 3 deficiencyleads to abnormal sarcomeres, impairment of muscle contractile capacity, and death of the muscle fibers. These data indicate a ... Kroupova I, VondracekP, Marikova T, Mazanec R, Zamecnik J, Stanek J,Havlova M & Fajkusova L (2004) Mutations in CzechLGMD 2A patients revealed by analysis of calpain3 mRNA and their phenotypic ... activity of calpain 3As a cytoplasmic protease, the activity of calpain 3must be tightly regulated temporally and spatially tobe effective and to avoid unwanted damage. Besidescontrol at the transcriptional...