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Calpain 3: a key regulator of the sarcomere?

Abstract : Calpain 3 is a 94-kDa calcium-dependent cysteine protease mainly expressed in skeletal muscle. In this tissue, it localizes at several regions of the sarcomere through binding to the giant protein, titin. Loss-of-function mutations in the calpain 3 gene have been associated with limb-girdle muscular dystrophy type 2A (LGMD2A), a common form of muscular dystrophy found world wide. Recently, significant progress has been made in understanding the mode of regulation and the possible function of calpain 3 in muscle. It is now well accepted that it has an unusual zymogenic activation and that cytoskeletal proteins are one class of its substrates. Through the absence of cleavage of these substrates, calpain 3 deficiency leads to abnormal sarcomeres, impairment of muscle contractile capacity, and death of the muscle fibers. These data indicate a role for calpain 3 as a chef d'orchestre in sarcomere remodeling and suggest a new category of LGMD2 pathological mechanisms.
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Contributor : Marc Bartoli <>
Submitted on : Wednesday, October 4, 2017 - 12:22:12 PM
Last modification on : Thursday, March 4, 2021 - 3:50:24 PM

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Marc Bartoli, Stéphanie Duguez, Isabelle Richard. Calpain 3: a key regulator of the sarcomere?. FEBS Journal, Wiley, 2006, 273 (15), pp.3427-3436. ⟨10.1111/j.1742-4658.2006.05351.x⟩. ⟨hal-01610048⟩



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