Missense mutation of c.635 T > C in CAPN3 impairs muscle injury repair in a Limb-Girdel Muscular Dystropy Model. | Missense mutation of c.635 T > C in CAPN3 impairs muscle injury repair in a Limb-Girdel Muscular Dystropy Model. Ma HS, Gong XL, Li WX, Cai Q, Chen YW, Guo XB, Ren ZR, Zeng F, Yan JB. | 05/19/2023 |
A Small-Molecule Approach to Restore a Slow-Oxidative Phenotype and Defective CaMKIIbeta Signaling in Limb Girdle Muscular Dystrophy. | A Small-Molecule Approach to Restore a Slow-Oxidative Phenotype and Defective CaMKIIβ Signaling in Limb Girdle Muscular Dystrophy. Liu J, Campagna J, John V, Damoiseaux R, Mokhonova E, Becerra D, Meng H, McNally EM, Pyle AD, Kramerova I, Spencer MJ., Free PMC Article | 02/26/2022 |
Mitochondrial dysfunction and consequences in calpain-3-deficient muscle. | Mitochondrial dysfunction and consequences in calpain-3-deficient muscle. Jahnke VE, Peterson JM, Van Der Meulen JH, Boehler J, Uaesoontrachoon K, Johnston HK, Defour A, Phadke A, Yu Q, Jaiswal JK, Nagaraju K., Free PMC Article | 10/9/2021 |
Frizzled related protein deficiency impairs muscle strength, gait and calpain 3 levels. | Frizzled related protein deficiency impairs muscle strength, gait and calpain 3 levels. Casas-Fraile L, Cornelis FM, Costamagna D, Rico A, Duelen R, Sampaolesi MM, López de Munain A, Lories RJ, Sáenz A., Free PMC Article | 07/31/2021 |
Study shows that impaired skeletal muscle regeneration in calpain-3 null muscle is associated with perturbations in mTORC1 signaling and defective mitochondrial biogenesis. | Impaired regeneration in calpain-3 null muscle is associated with perturbations in mTORC1 signaling and defective mitochondrial biogenesis. Yalvac ME, Amornvit J, Braganza C, Chen L, Hussain SA, Shontz KM, Montgomery CL, Flanigan KM, Lewis S, Sahenk Z., Free PMC Article | 09/29/2018 |
Destabilization of PGC1a is attributable to decreased p38 MAPK activation via diminished CaMKII signaling. Thus, we elucidate a pathway downstream of Ca(2+)-mediated CaMKII activation that is dysfunctional in C3KO(Capn3 knock-out mice ) mice, leading to reduced transcription of genes involved in muscle adaptation | Failure to up-regulate transcription of genes necessary for muscle adaptation underlies limb girdle muscular dystrophy 2A (calpainopathy). Kramerova I, Ermolova N, Eskin A, Hevener A, Quehenberger O, Armando AM, Haller R, Romain N, Nelson SF, Spencer MJ., Free PMC Article | 09/30/2017 |
CAPN3 deficiency leads to degradation of SERCA proteins and Ca2+ dysregulation in the skeletal muscle. | Calpain 3 deficiency affects SERCA expression and function in the skeletal muscle. Toral-Ojeda I, Aldanondo G, Lasa-Elgarresta J, Lasa-Fernández H, Fernández-Torrón R, López de Munain A, Vallejo-Illarramendi A., Free PMC Article | 10/22/2016 |
Cleavage of C-terminal titin by CAPN3 is associated with limb-girdle muscular dystrophy 2A and tibial muscular dystrophy. | CAPN3-mediated processing of C-terminal titin replaced by pathological cleavage in titinopathy. Charton K, Sarparanta J, Vihola A, Milic A, Jonson PH, Suel L, Luque H, Boumela I, Richard I, Udd B. | 04/30/2016 |
these studies reveal a novel interaction between CAPN3 and CaM and identify CaM as the first positive regulator of CAPN3 activity. | Autolytic activation of calpain 3 proteinase is facilitated by calmodulin protein. Ermolova N, Kramerova I, Spencer MJ., Free PMC Article | 04/25/2015 |
data suggest that skNAC controls myoblast migration and sarcomere architecture in a calpain-dependent manner | skNAC depletion stimulates myoblast migration and perturbs sarcomerogenesis by enhancing calpain 1 and 3 activity. Berkholz J, Zakrzewicz A, Munz B. | 09/21/2013 |
our results suggest that a component of FSHD pathogenesis may arise by over-expression of FRG1, reducing Rbfox1 levels and leading to aberrant expression of an altered Calpain 3 protein through dysregulated splicing | Rbfox1 downregulation and altered calpain 3 splicing by FRG1 in a mouse model of Facioscapulohumeral muscular dystrophy (FSHD). Pistoni M, Shiue L, Cline MS, Bortolanza S, Neguembor MV, Xynos A, Ares M Jr, Gabellini D., Free PMC Article | 06/1/2013 |
The Ky gene was downregulated in CAPN3 knockout muscles suggesting that Ky protease may play a complementary role in regulating muscle cytoskeleton homeostasis in response to changes in muscle activity | C3KO mouse expression analysis: downregulation of the muscular dystrophy Ky protein and alterations in muscle aging. Jaka O, Kramerova I, Azpitarte M, López de Munain A, Spencer M, Sáenz A. | 04/13/2013 |
The decrease in CaMKII signaling in the absence of CAPN3 is associated with a reduction of muscle adaptation response. | Impaired calcium calmodulin kinase signaling and muscle adaptation response in the absence of calpain 3. Kramerova I, Kudryashova E, Ermolova N, Saenz A, Jaka O, López de Munain A, Spencer MJ., Free PMC Article | 01/26/2013 |
stretch-induced dynamic redistribution of p94 is dependent on its protease activity and essential to protect muscle from degeneration | Dynamic distribution of muscle-specific calpain in mice has a key role in physical-stress adaptation and is impaired in muscular dystrophy. Ojima K, Kawabata Y, Nakao H, Nakao K, Doi N, Kitamura F, Ono Y, Hata S, Suzuki H, Kawahara H, Bogomolovas J, Witt C, Ottenheijm C, Labeit S, Granzier H, Toyama-Sorimachi N, Sorimachi M, Suzuki K, Maeda T, Abe K, Aiba A, Sorimachi H., Free PMC Article | 09/6/2010 |
roles for Na(+) dependence of p94 | Skeletal muscle-specific calpain is an intracellular Na+-dependent protease. Ono Y, Ojima K, Torii F, Takaya E, Doi N, Nakagawa K, Hata S, Abe K, Sorimachi H., Free PMC Article | 08/23/2010 |
In vitro experiments have then revealed that only PDLIM1 is cleaved directly by calpain-3. | A proteomic study of calpain-3 and its involvement in limb girdle muscular dystrophy type 2a. Bertipaglia I, Bourg N, Richard I, Pahlman AK, Andersson L, James P, Carafoli E. | 08/2/2010 |
Mitochondrial abnormalities in the skeletal muscle of calpain 3 knockout mice correlate with the presence of oxidative stress. | Mitochondrial abnormalities, energy deficit and oxidative stress are features of calpain 3 deficiency in skeletal muscle. Kramerova I, Kudryashova E, Wu B, Germain S, Vandenborne K, Romain N, Haller RG, Verity MA, Spencer MJ., Free PMC Article | 01/21/2010 |
calpain 3 is uniquely activated during lens fiber differentiation. | Calpain expression and activity during lens fiber cell differentiation. De Maria A, Shi Y, Kumar NM, Bassnett S., Free PMC Article | 01/21/2010 |
calpain-3 is not responsible for Ca(2+)-induced disruption of EC coupling, but mu-calpain is a plausible candidate | Involvement of calpains in Ca2+-induced disruption of excitation-contraction coupling in mammalian skeletal muscle fibers. Verburg E, Murphy RM, Richard I, Lamb GD. | 01/21/2010 |
CAPN3 to be necessary for recruitment of AldoA to one specific location, namely the triads, which are structural components of muscle responsible for calcium transport and excitation-contraction coupling | Novel role of calpain-3 in the triad-associated protein complex regulating calcium release in skeletal muscle. Kramerova I, Kudryashova E, Wu B, Ottenheijm C, Granzier H, Spencer MJ., Free PMC Article | 01/21/2010 |
implicate the dynamic nature of connectin molecule as a regulatory scaffold of p94 functions | Multiple molecular interactions implicate the connectin/titin N2A region as a modulating scaffold for p94/calpain 3 activity in skeletal muscle. Hayashi C, Ono Y, Doi N, Kitamura F, Tagami M, Mineki R, Arai T, Taguchi H, Yanagida M, Hirner S, Labeit D, Labeit S, Sorimachi H. | 01/21/2010 |
calpain 3 is necessary for ubiquitination and that it acts upstream of the ubiquitination machinery | Calpain 3 participates in sarcomere remodeling by acting upstream of the ubiquitin-proteasome pathway. Kramerova I, Kudryashova E, Venkatraman G, Spencer MJ. | 10/1/2016 |
the Capn3 activation mechanism is similar to the universal activation of caspases and corresponds to an autolysis within the active site of the protease | Calpain 3 is activated through autolysis within the active site and lyses sarcomeric and sarcolemmal components. Taveau M, Bourg N, Sillon G, Roudaut C, Bartoli M, Richard I., Free PMC Article | 01/21/2010 |
C/EBP alpha is required for cleavage of cyclin A by calpain 3 in myeloid precursor cells. | C/EBPalpha is required for proteolytic cleavage of cyclin A by calpain 3 in myeloid precursor cells. Welm AL, Timchenko NA, Ono Y, Sorimachi H, Radomska HS, Tenen DG, Lekstrom-Himes J, Darlington GJ. | 01/21/2010 |
role in muscle maturation | 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. Spencer MJ, Guyon JR, Sorimachi H, Potts A, Richard I, Herasse M, Chamberlain J, Dalkilic I, Kunkel LM, Beckmann JS., Free PMC Article | 01/21/2010 |