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.

Baghdiguian, S; Martin, M; Richard, I; Pons, F; Astier, C; Bourg, N; Hay, R T; Chemaly, R; Halaby, G; Loiselet, J; Anderson, L V; Lopez de Munain, A; Fardeau, M; Mangeat, P; Beckmann, J S; Lefranc, G

Baghdiguian, S; Martin, M; Richard, I; Pons, F; Astier, C; Bourg, N; Hay, R T; Chemaly, R; Halaby, G; Loiselet, J; Anderson, L V; Lopez de Munain, A; Fardeau, M; Mangeat, P; Beckmann, J S; Lefranc, G.

Affiliation

Baghdiguian S; Laboratoire de Dynamique Moléculaire des Interactions Membranaires, CNRS-UMR 5539, Université Montpellier II, France.

Nat Med ; 5(5): 503-11, 1999 May.

Article in En | MEDLINE | ID: mdl-10229226

Subject(s)

Apoptosis; Calpain/deficiency; Cell Nucleus/pathology; DNA-Binding Proteins/metabolism; I-kappa B Proteins; Muscular Dystrophies/metabolism; NF-kappa B/metabolism; Adolescent; Adult; Biological Transport; Calpain/metabolism; Cell Compartmentation; Child; DNA-Binding Proteins/genetics; Female; Fetus; Fluorescent Antibody Technique; Humans; Male; Microscopy, Confocal; Muscle, Skeletal/metabolism; Muscle, Skeletal/pathology; Muscular Dystrophies/classification; Muscular Dystrophies/enzymology; Muscular Dystrophies/pathology; NF-KappaB Inhibitor alpha; NF-kappa B/antagonists & inhibitors; Recombinant Proteins/metabolism

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Database: MEDLINE Main subject: Calpain / Cell Nucleus / NF-kappa B / Apoptosis / I-kappa B Proteins / DNA-Binding Proteins / Muscular Dystrophies Type of study: Risk_factors_studies Limits: Adolescent / Adult / Child / Female / Humans / Male Language: En Year: 1999 Type: Article

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