The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Loss of Calpain 3 Proteolytic Activity Leads to Muscular Dystrophy and to Apoptosis-Associated Iκbα/Nuclear Factor κb Pathway Perturbation in Mice
Open accessAbstract
Calpain 3 is known as the skeletal muscle–specific member of the calpains, a family of intracellular nonlysosomal cysteine proteases. It was previously shown that defects in the human calpain 3 gene are responsible for limb girdle muscular dystrophy type 2A (LGMD2A), an inherited disease affecting predominantly the proximal limb muscles. To better understand the function of calpain 3 and the pathophysiological mechanisms of LGMD2A and also to develop an adequate model for therapy research, we generated capn3-deficient mice by gene targeting. capn3-deficient mice are fully fertile and viable. Allele transmission in intercross progeny demonstrated a statistically significant departure from Mendel's law. capn3-deficient mice show a mild progressive muscular dystrophy that affects a specific group of muscles. The age of appearance of myopathic features varies with the genetic background, suggesting the involvement of modifier genes. Affected muscles manifest a similar apoptosis-associated perturbation of the IκBα/nuclear factor κB pathway as seen in LGMD2A patients. In addition, Evans blue staining of muscle fibers reveals that the pathological process due to calpain 3 deficiency is associated with membrane alterations.
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Cites
Animal models for muscular dystrophy show different patterns of sarcolemmal disruption
Straub, V., Rafael, J. A., Chamberlain, J. S., & Campbell, K. P. (1997) · The Journal of Cell Biology · doi:10.1083/jcb.139.2.375 · Cited by 488
Cited by
Nuclear factor-?B: its role in health and disease
Kumar, A., Takada, Y., Boriek, A., & Aggarwal, B. (2004) · Journal of Molecular Medicine · doi:10.1007/s00109-004-0555-y · Cited by 933
The ubiquitin-proteasome system
Nandi, D., Tahiliani, P., Kumar, A., & Chandu, D. (2006) · Journal of Biosciences · doi:10.1007/bf02705243 · Cited by 758
Du, J., Wang, X., Miereles, C., Bailey, J. L., Debigare, R., Zheng, B., Price, S. R., & Mitch, W. E. (2004) · Journal of Clinical Investigation · doi:10.1172/jci18330 · Cited by 731
Hackman, P., Vihola, A., Haravuori, H., Marchand, S., Sarparanta, J., de Seze, J., Labeit, S., Witt, C., Peltonen, L., Richard, I., et al. (2002) · The American Journal of Human Genetics · doi:10.1086/342380 · Cited by 473
Self-citationNuclear factor-kappa B signaling in skeletal muscle atrophy
Li, H., Malhotra, S., & Kumar, A. (2008) · Journal of Molecular Medicine · doi:10.1007/s00109-008-0373-8 · Cited by 441
Metadata
- Authors
- Richard, I., Roudaut, C., Marchand, S., Baghdiguian, S., Herasse, M., Stockholm, D., Ono, Y., Suel, L., Bourg, N., Sorimachi, H., Lefranc, G., Fardeau, M., Sébille, A., & Beckmann, J. S.
- Venue
- The Journal of Cell Biology
- Publisher
- Rockefeller University Press
- Published
- 2000-12-25
- Volume
- 151
- Issue
- 7
- Pages
- 1583-1590
- ISSN
- 0021-9525 (ISSN-L)
- 1540-8140
- OpenAlex
- W2133145432
- Cited by
- 177
- References
- 1
- FWCI
- 3.20
- Type
- journal article
- Language
- en
- Open access
- green
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- CoChat. (2026). Citation check: Loss of Calpain 3 Proteolytic Activity Leads to Muscular Dystrophy and to Apoptosis-Associated Iκbα/Nuclear Factor κb Pathway Perturbation in Mice. https://app.cochat.ai/papers/10.1083/jcb.151.7.1583
- The paper
- Richard, I., Roudaut, C., Marchand, S., Baghdiguian, S., Herasse, M., Stockholm, D., Ono, Y., Suel, L., Bourg, N., Sorimachi, H., Lefranc, G., Fardeau, M., Sébille, A., & Beckmann, J. S. (2000). Loss of Calpain 3 Proteolytic Activity Leads to Muscular Dystrophy and to Apoptosis-Associated Iκbα/Nuclear Factor κb Pathway Perturbation in Mice. The Journal of Cell Biology. https://doi.org/10.1083/jcb.151.7.1583
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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