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• W2022972335 abstract "Limb-girdle muscular dystrophy type 2A (LGMD2A, also called “calpainopathy”) is caused by defects in p94/calpain 3, which is the skeletal muscle-specific member of Ca2+-dependent cysteine protease “calpain”. Mammals have 15 genes for calpains (CAPN1 ∼ CAPN3, CAPN5 ∼ CAPN16), which are involved in a variety of cellular functions, and p94 is encoded by CAPN3. Particularly, mouse gene knock-out of Capn2, which encodes catalytic subunit of ubiquitous m-calpain, causes embryonic lethality, and that of Capn3 results in calpainopathy in mice, indicating an essential role of calpain in mammalian life and health. However, the mechanism of p94’s function in the pathogenesis of calpainopathy remains unclear. Here, we demonstrate that the stretch-dependent p94 distribution plays a crucial role in the pathogenesis of calpainopathy, using p94:C129S “knock-in” (p94CS-KI) mice, in which the endogenous p94 was replaced with a proteolytically inactive but structurally intact p94:C129S mutant protein. The p94CS-KI mice developed a progressive muscular dystrophy, which was exacerbated by ageing or exercise, although their phenotype (myofibril integrity, serum CK levels, etc.) is less severe than that of p94 knock-out (p94KO) mice. The exercise-induced muscle degeneration in p94CS-KI mice was associated with an inefficient redistribution of p94:C129S in stretched sarcomeres. Furthermore, the p94CS-KI mice showed impaired adaptation to physical stress, accompanied by an exercise-dependent alteration in the muscle ankyrin-repeat protein-2 (MARP2) level. These findings indicate that the stretch-induced dynamic redistribution of p94 is affected by its protease activity and is essential to protect skeletal muscle cells from degeneration, particularly under physical stress." @default.
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• W2022972335 date "2010-10-01" @default.
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• W2022972335 title "O.5 Skeletal muscle-specific calpain, p94/calpain 3, dynamically distributes in skeletal muscle cells to adapt to physical stress, defects of which cause muscular dystrophy" @default.
• W2022972335 doi "https://doi.org/10.1016/j.nmd.2010.07.014" @default.
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