• 1
    Goll DE, Thompson VF, Li H, Wei W & Cong J (2003) The calpain system. Physiol Rev 83, 731801.
  • 2
    Richard I, Broux O, Allamand V, Fougerousse F, Chiannilkulchai N, Bourg N, Brenguier L, Devaud C, Pasturaud P, Roudaut C, et al. (1995) Mutations in the proteolytic enzyme calpain 3 cause limb-girdle muscular dystrophy type 2A. Cell 81, 2740.
  • 3
    Urtasun M, Saenz A, Roudaut C, Poza JJ, Urtizberea JA, Cobo AM, Richard I, Garcia Bragado F, Leturcq F, Kaplan JC, et al. (1998) Limb-girdle muscular dystrophy in Guipuzcoa (Basque Country, Spain). Brain 121, 17351747.
  • 4
    Fanin M, Nascimbeni AC, Fulizio L & Angelini C (2005) The frequency of limb girdle muscular dystrophy 2A in northeastern Italy. Neuromuscul Disord 15, 218224.
  • 5
    Fardeau M, Eymard B, Mignard C, Tome FM, Richard I & Beckmann JS (1996) Chromosome 15-linked limb-girdle muscular dystrophy: clinical phenotypes in Reunion Island and French metropolitan communities. Neuromuscul Disord 6, 447453.
  • 6
    Sorimachi H, Imajoh-Ohmi S, Emori Y, Kawasaki H, Ohno S, Minami Y & Suzuki K (1989) Molecular cloning of a novel mammalian calcium-dependent protease distinct from both m- and mu-types. Specific expression of the mRNA in skeletal muscle. J Biol Chem 264, 2010620111.
  • 7
    Jenne DE, Kley RA, Vorgerd M, Schroder JM, Weis J, Reimann H, Albrecht B, Nurnberg P, Thiele H, Muller CR, et al. (2005) Limb girdle muscular dystrophy in a sibling pair with a homozygous Ser606Leu mutation in the alternatively spliced IS2 region of calpain 3. Biol Chem 386, 6167.
  • 8
    Herasse M, Ono Y, Fougerousse F, Kimura E, Stockholm D, Beley C, Montarras D, Pinset C, Sorimachi H, Suzuki K, et al. (1999) Expression and functional characteristics of calpain 3 isoforms generated through tissue-specific transcriptional and posttranscriptional events. Mol Cell Biol 19, 40474055.
  • 9
    Kawabata Y, Hata S, Ono Y, Ito Y, Suzuki K, Abe K & Sorimachi H (2003) Newly identified exons encoding novel variants of p94/calpain 3 are expressed ubiquitously and overlap the alpha-glucosidase C gene. FEBS Lett 555, 623630.
  • 10
    Jia Z, Petrounevitch V, Wong A, Moldoveanu T, Davies PL, Elce JS & Beckmann JS (2001) Mutations in calpain 3 associated with limb girdle muscular dystrophy: analysis by molecular modeling and by mutation in m-calpain. Biophys J 80, 25902596.
  • 11
    Suzuki K & Sorimachi H (1998) A novel aspect of calpain activation. FEBS Lett 433, 14.
  • 12
    Sorimachi H, Toyama-Sorimachi N, Saido TC, Kawasaki H, Sugita H, Miyasaka M, Arahata K, Ishiura S & Suzuki K (1993) Muscle-specific calpain, p94, is degraded by autolysis immediately after translation, resulting in disappearance from muscle. J Biol Chem 268, 1059310605.
  • 13
    Taveau M, Bourg N, Sillon G, Roudaut C, Bartoli M & Richard I (2003) Calpain 3 is activated through autolysis within the active site and lyses sarcomeric and sarcolemmal components. Mol Cell Biol 23, 91279135.
  • 14
    Baghdiguian S, Martin M, Richard I, Pons F, Astier C, Bourg N, Hay RT, Chemaly R, Halaby G, Loiselet J, et al. (1999) Calpain 3 deficiency is associated with myonuclear apoptosis and profound perturbation of the IkappaB alpha/NF-kappaB pathway in limb-girdle muscular dystrophy type 2A. Nat Med 5, 503511.
  • 15
    Ono Y, Shimada H, Sorimachi H, Richard I, Saido TC, Beckmann JS, Ishiura S & Suzuki K (1998) Functional defects of a muscle-specific calpain, p94, caused by mutations associated with limb-girdle muscular dystrophy type 2A. J Biol Chem 273, 1707317078.
  • 16
    Diaz BG, Moldoveanu T, Kuiper MJ, Campbell RL & Davies PL (2004) Insertion sequence 1 of muscle-specific calpain, p94, acts as an internal propeptide. J Biol Chem 279, 2765627666.
  • 17
    Ravulapalli R, Diaz BG, Campbell RL & Davies PL (2005) Homodimerization of calpain 3 penta-EF-hand domain. Biochem J 388, 585591.
  • 18
    Graham-Siegenthaler K, Gauthier S, Davies PL & Elce JS (1994) Active recombinant rat calpain II. Bacterially produced large and small subunits associate both in vivo and in vitro. J Biol Chem 269, 3045730460.activity and a novel mode of enzyme activation. EMBO J 18, 6880–6889.
  • 19
    Hosfield CM, Elce JS, Davies PL & Jia Z (1999) Crystal structure of calpain reveals the structural basis for Ca(2+)-dependent protease
  • 20
    Moldoveanu T, Hosfield CM, Lim D, Elce JS, Jia Z & Davies PL (2002) A Ca(2+) switch aligns the active site of calpain. Cell 108, 649660.
  • 21
    Kinbara K, Sorimachi H, Ishiura S & Suzuki K (1997) Muscle-specific calpain, p94, interacts with the extreme C-terminal region of connectin, a unique region flanked by two immunoglobulin C2 motifs. Arch Biochem Biophys 342, 99107.
  • 22
    Sorimachi H, Kinbara K, Kimura S, Takahashi M, Ishiura S, Sasagawa N, Sorimachi N, Shimada H, Tagawa K, Maruyama K, et al. (1995) Muscle-specific calpain, p94, responsible for limb girdle muscular dystrophy type 2A, associates with connectin through IS2, a p94-specific sequence. J Biol Chem 270, 3115831162.
  • 23
    Horowits R (1999) The physiological role of titin in striated muscle. Rev Physiol Biochem Pharmacol 138, 5796.
  • 24
    Keira Y, Noguchi S, Minami N, Hayashi YK & Nishino I (2003) Localization of calpain 3 in human skeletal muscle and its alteration in limb-girdle muscular dystrophy 2A muscle. J Biochem (Tokyo) 133, 659664.
  • 25
    Anderson LV, Davison K, Moss JA, Richard I, Fardeau M, Tome FM, Hubner C, Lasa A, Colomer J & Beckmann JS (1998) Characterization of monoclonal antibodies to calpain 3 and protein expression in muscle from patients with limb-girdle muscular dystrophy type 2A. Am J Pathol 153, 11691179.
  • 26
    Bartoli M, Roudaut C, Martin S, Fougerousse F, Suel L, Poupiot J, Gicquel E, Noulet F, Danos O & Richard I (2006) Safety and efficacy of AAV-mediated calpain 3 gene transfer in a mouse model of limb-girdle muscular dystrophy type 2A. Mol Ther 13, 250259.
  • 27
    Spencer MJ, Guyon JR, Sorimachi H, Potts A, Richard I, Herasse M, Chamberlain J, Dalkilic I, Kunkel LM & Beckmann JS (2002) Stable expression of calpain 3 from a muscle transgene in vivo: immature muscle in transgenic mice suggests a role for calpain 3 in muscle maturation. Proc Natl Acad Sci USA 99, 88748879.
  • 28
    Garvey SM, Rajan C, Lerner AP, Frankel WN & Cox GA (2002) The muscular dystrophy with myositis (mdm) mouse mutation disrupts a skeletal muscle-specific domain of titin. Genomics 79, 146149.
  • 29
    Huebsch KA, Kudryashova E, Wooley CM, Sher RB, Seburn KL, Spencer MJ & Cox GA (2005) mdm muscular dystrophy: interactions with calpain 3 and a novel functional role for titin's N2A domain. Hum Mol Genet 14, 28012811.
  • 30
    Kinbara K, Ishiura S, Tomioka S, Sorimachi H, Jeong SY, Amano S, Kawasaki H, Kolmerer B, Kimura S, Labeit S, et al. (1998) Purification of native p94, a muscle-specific calpain, and characterization of its autolysis. Biochem J 335, 589596.
  • 31
    Maruyama K, Endo T, Kume H, Kawamura Y, Kanzawa N, Nakauchi Y, Kimura S, Kawashima S & Maruyama K (1993) A novel domain sequence of connectin localized at the I band of skeletal muscle sarcomeres: homology to neurofilament subunits. Biochem Biophys Res Commun 194, 1288.
  • 32
    Haravuori H, Vihola A, Straub V, Auranen M, Richard I, Marchand S, Voit T, Labeit S, Somer H, Peltonen L, et al. (2001) Secondary calpain3 deficiency in 2q-linked muscular dystrophy: titin is the candidate gene. Neurology 56, 869877.
  • 33
    Hackman P, Vihola A, Haravuori H, Marchand S, Sarparanta J, De Seze J, Labeit S, Witt C, Peltonen L, Richard I & Udd B (2002) Tibial muscular dystrophy is a titinopathy caused by mutations in TTN, the gene encoding the giant skeletal-muscle protein titin. Am J Hum Genet 71, 492500.
  • 34
    Fanin M, Nascimbeni AC, Fulizio L, Trevisan CP, Meznaric-Petrusa M & Angelini C (2003) Loss of calpain-3 autocatalytic activity in LGMD2A patients with normal protein expression. Am J Pathol 163, 19291936.
  • 35
    Rey MA & Davies PL (2002) The protease core of the muscle-specific calpain, p94, undergoes Ca2+-dependent intramolecular autolysis. FEBS Lett 532, 401406.
  • 36
    Garcia Diaz BE, Gauthier S & Davies PL (2006) Ca(2+) dependency of calpain 3 (p94) activation. Biochemistry 45, 37143722.
  • 37
    Feasson L, Stockholm D, Freyssenet D, Richard I, Duguez S, Beckmann JS & Denis C (2002) Molecular adaptations of neuromuscular disease-associated proteins in response to eccentric exercise in human skeletal muscle. J Physiol 543, 297306.
  • 38
    Murphy RM, Snow RJ & Lamb GD (2006) mu-calpain and calpain-3 are not autolyzed with exhaustive exercise in humans. Am J Physiol Cell Physiol 290, C116C122.
  • 39
    Guyon JR, Kudryashova E, Potts A, Dalkilic I, Brosius MA, Thompson TG, Beckmann JS, Kunkel LM & Spencer MJ (2003) Calpain 3 cleaves filamin C and regulates its ability to interact with gamma- and delta-sarcoglycans. Muscle Nerve 28, 472483.
  • 40
    Kramerova I, Kudryashova E, Tidball JG & Spencer MJ (2004) Null mutation of calpain 3 (p94) in mice causes abnormal sarcomere formation in vivo and in vitro. Hum Mol Genet 13, 13731388.
  • 41
    Hayashi M, Suzuki H, Kawashima S, Saido TC & Inomata M (1999) The behavior of calpain-generated N- and C-terminal fragments of talin in integrin-mediated signaling pathways. Arch Biochem Biophys 371, 133141.
  • 42
    Yan B, Calderwood DA, Yaspan B & Ginsberg MH (2001) Calpain cleavage promotes talin binding to the beta 3 integrin cytoplasmic domain. J Biol Chem 276, 2816428170.
  • 43
    Fougerousse F, Durand M, Suel L, Pourquie O, Delezoide AL, Romero NB, Abitbol M & Beckmann JS (1998) Expression of genes (CAPN3, SGCA, SGCB, and TTN) involved in progressive muscular dystrophies during early human development. Genomics 48, 145156.
  • 44
    Stockholm D, Herasse M, Marchand S, Praud C, Roudaut C, Richard I, Sebille A & Beckmann JS (2001) Calpain 3 mRNA expression in mice after denervation and during muscle regeneration. Am J Physiol Cell Physiol 280, C1561C1569.
  • 45
    Miyabara EH, Aoki MS, Soares AG & Moriscot AS (2005) Expression of tropism-related genes in regenerating skeletal muscle of rats treated with cyclosporin-A. Cell Tissue Res 319, 479489.
  • 46
    Nakashima K, Yamazaki M & Abe H (2005) Effects of serum deprivation on expression of proteolytic-related genes in chick myotube cultures. Biosci Biotechnol Biochem 69, 623627.
  • 47
    Busquets S, Garcia-Martinez C, Alvarez B, Carbo N, Lopez-Soriano FJ & Argiles JM (2000) Calpain-3 gene expression is decreased during experimental cancer cachexia. Biochim Biophys Acta 1475, 59.
  • 48
    Tsujinaka T, Fujita J, Ebisui C, Yano M, Kominami E, Suzuki K, Tanaka K, Katsume A, Ohsugi Y, Shiozaki H, et al. (1996) Interleukin 6 receptor antibody inhibits muscle atrophy and modulates proteolytic systems in interleukin 6 transgenic mice. J Clin Invest 97, 244249.
  • 49
    Bartoli M & Richard I (2005) Calpains in muscle wasting. Int J Biochem Cell Biol 37, 21152133.
  • 50
    Lapidos KA, Kakkar R & McNally EM (2004) The dystrophin glycoprotein complex: signaling strength and integrity for the sarcolemma. Circ Res 94, 10231031.
  • 51
    Vainzof M, de Paula F, Tsanaclis AM & Zatz M (2003) The effect of calpain 3 deficiency on the pattern of muscle degeneration in the earliest stages of LGMD2A. J Clin Pathol 56, 624626.
  • 52
    Chrobakova T, Hermanova M, Kroupova I, Vondracek P, Marikova T, Mazanec R, Zamecnik J, Stanek J, Havlova M & Fajkusova L (2004) Mutations in Czech LGMD2A patients revealed by analysis of calpain3 mRNA and their phenotypic outcome. Neuromuscul Disord 14, 659665.
  • 53
    Pollitt C, Anderson LV, Pogue R, Davison K, Pyle A & Bushby KM (2001) The phenotype of calpainopathy: diagnosis based on a multidisciplinary approach. Neuromuscul Disord 11, 287296.
  • 54
    Richard I, Roudaut C, Marchand S, Baghdiguian S, Herasse M, Stockholm D, Ono Y, Suel L, Bourg N, Sorimachi H, et al. (2000) Loss of calpain 3 proteolytic activity leads to muscular dystrophy and to apoptosis-associated IkappaBalpha/nuclear factor kappaB pathway perturbation in mice. J Cell Biol 151, 15831590.
  • 55
    Fougerousse F, Gonin P, Durand M, Richard I & Raymackers JM (2003) Force impairment in calpain 3-deficient mice is not correlated with mechanical disruption. Muscle Nerve 27, 616623.
  • 56
    Bansal D, Miyake K, Vogel SS, Groh S, Chen CC, Williamson R, McNeil PL & Campbell KP (2003) Defective membrane repair in dysferlin-deficient muscular dystrophy. Nature 423, 168172.
  • 57
    Bashir R, Britton S, Strachan T, Keers S, Vafiadaki E, Lako M, Richard I, Marchand S, Bourg N, Argov Z, et al. (1998) A gene related to Caenorhabditis elegans spermatogenesis factor fer-1 is mutated in limb-girdle muscular dystrophy type 2B. Nat Genet 20, 3742.
  • 58
    Huang Y, Verheesen P, Roussis A, Frankhuizen W, Ginjaar I, Haldane F, Laval S, Anderson LV, Verrips T, Frants RR, et al. (2005) Protein studies in dysferlinopathy patients using llama-derived antibody fragments selected by phage display. Eur J Hum Genet 13, 721730.
  • 59
    Chae J, Minami N, Jin Y, Nakagawa M, Murayama K, Igarashi F & Nonaka I (2001) Calpain 3 gene mutations: genetic and clinico-pathologic findings in limb-girdle muscular dystrophy. Neuromuscul Disord 11, 547555.
  • 60
    Poussard S, Duvert M, Balcerzak D, Ramassamy S, Brustis JJ, Cottin P & Ducastaing A (1996) Evidence for implication of muscle-specific calpain (p94) in myofibrillar integrity. Cell Growth Differ 7, 14611469.
  • 61
    Attaix D, Mosoni L, Dardevet D, Combaret L, Mirand PP & Grizard J (2005) Altered responses in skeletal muscle protein turnover during aging in anabolic and catabolic periods. Int J Biochem Cell Biol 37, 19621973.
  • 62
    Huang J & Forsberg NE (1998) Role of calpain in skeletal-muscle protein degradation. Proc Natl Acad Sci USA 95, 1210012105.
  • 63
    Cao PR, Kim HJ & Lecker SH (2005) Ubiquitin-protein ligases in muscle wasting. Int J Biochem Cell Biol 37, 20882097.
  • 64
    Kramerova I, Kudryashova E, Venkatraman G & Spencer MJ (2005) Calpain 3 participates in sarcomere remodeling by acting upstream of the ubiquitin-proteasome pathway. Hum Mol Genet 14, 21252134.
  • 65
    Combaret L, Bechet D, Claustre A, Taillandier D, Richard I & Attaix D (2003) Down-regulation of genes in the lysosomal and ubiquitin-proteasome proteolytic pathways in calpain-3-deficient muscle. Int J Biochem Cell Biol 35, 676684.