Calpain 3 deficiency is associated with myonuclear apoptosis and profound perturbation of the IκBα/NF-κB pathway in limb-girdle muscular dystrophy type 2A
Using fluorescence microscopy techniques, we show here that muscle biopsies of LGMD2A patients present apoptotic features that are associated with subsarcolemmal localization of NF- Kappa B and selective nuclear accumulation of I Kappa B alpha . Furthermore, we reconstituted calpain 3-dependent degr...
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Published in | Nature medicine Vol. 5; no. 5; pp. 503 - 511 |
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Main Authors | , , , , , , , , , , , , , , , |
Format | Journal Article |
Language | English |
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Nature Publishing Group US
01.05.1999
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Abstract | Using fluorescence microscopy techniques, we show here that muscle biopsies of LGMD2A patients present apoptotic features that are associated with subsarcolemmal localization of NF- Kappa B and selective nuclear accumulation of I Kappa B alpha . Furthermore, we reconstituted calpain 3-dependent degradation of I Kappa B alpha in an in vitro cell expression system, and I Kappa B alpha -dependent apoptosis in human myogenic satellite cells grown in primary culture. Thus, the apoptotic cell death event in LGMD2A is compatible with a sequence of events involving a failure in calpain 3-dependent I Kappa B alpha proteolysis resulting in I Kappa B alpha accumulation in both the cytoplasm and nucleus, and sequestration of NF- Kappa B in the cytoplasm and subsequent non-expression of survival genes. |
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AbstractList | Using fluorescence microscopy techniques, we show here that muscle biopsies of LGMD2A patients present apoptotic features that are associated with subsarcolemmal localization of NF- Kappa B and selective nuclear accumulation of I Kappa B alpha . Furthermore, we reconstituted calpain 3-dependent degradation of I Kappa B alpha in an in vitro cell expression system, and I Kappa B alpha -dependent apoptosis in human myogenic satellite cells grown in primary culture. Thus, the apoptotic cell death event in LGMD2A is compatible with a sequence of events involving a failure in calpain 3-dependent I Kappa B alpha proteolysis resulting in I Kappa B alpha accumulation in both the cytoplasm and nucleus, and sequestration of NF- Kappa B in the cytoplasm and subsequent non-expression of survival genes. |
Author | Bourg, Nathalie Baghdiguian, Stephen Richard, Isabelle Munain, Adolfo Lopez de Lefranc, Gérard Pons, Françoise Chemaly, Raymond Mangeat, Paul Anderson, Louise V. B Hay, Ronald T Loiselet, Jacques Beckmann, Jacques S Martin, Marianne Astier, Catherine Fardeau, Michel Halaby, Georges |
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Snippet | Using fluorescence microscopy techniques, we show here that muscle biopsies of LGMD2A patients present apoptotic features that are associated with... |
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SubjectTerms | Biomedical and Life Sciences Biomedicine Cancer Research Infectious Diseases Metabolic Diseases Molecular Medicine Neurosciences |
Title | Calpain 3 deficiency is associated with myonuclear apoptosis and profound perturbation of the IκBα/NF-κB pathway in limb-girdle muscular dystrophy type 2A |
URI | http://dx.doi.org/10.1038/8385 https://link.springer.com/article/10.1038/8385 https://search.proquest.com/docview/17232137 |
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