Calpain 3 deficiency is associated with myonuclear apoptosis and profound perturbation of the IκBα/NF-κB pathway in limb-girdle muscular dystrophy type 2A
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The interaction of Piasy with Trim32, an E3-ubiquitin ligase mutated in limb-girdle muscular dystrophy type 2H, promotes Piasy degradation and regulates UVB-induced keratinocyte apoptosis through NFkappaBNovel variants of muscle calpain 3 identified in human melanoma cells: cisplatin-induced changes in vitro and differential expression in melanocytic lesionsTibial muscular dystrophy is a titinopathy caused by mutations in TTN, the gene encoding the giant skeletal-muscle protein titinPC4/Tis7/IFRD1 stimulates skeletal muscle regeneration and is involved in myoblast differentiation as a regulator of MyoD and NF-kappaBFilamin 2 (FLN2): A muscle-specific sarcoglycan interacting proteinThe familial Mediterranean fever protein, pyrin, is cleaved by caspase-1 and activates NF-kappaB through its N-terminal fragmentNF-kappaB, IkappaB, and IRAK control glutamate receptor density at the Drosophila NMJDistinctive patterns of microRNA expression in primary muscular disorders2-Benzamido-N-(1H-benzo[d]imidazol-2-yl)thiazole-4-carboxamide derivatives as potent inhibitors of CK1δ/εNovel role of calpain-3 in the triad-associated protein complex regulating calcium release in skeletal muscleDisruption of the murine calpain small subunit gene, Capn4: calpain is essential for embryonic development but not for cell growth and divisionDown-regulation of MyoD by calpain 3 promotes generation of reserve cells in C2C12 myoblastsCharacterization and expression of calpain 10. A novel ubiquitous calpain with nuclear localizationBAG3 deficiency results in fulminant myopathy and early lethalityThe lipid phosphatase myotubularin is essential for skeletal muscle maintenance but not for myogenesis in miceMuscular dystrophy begins early in embryonic development deriving from stem cell loss and disrupted skeletal muscle formationThe ichq mutant mouse, a model for the human skin disorder harlequin ichthyosis: mapping, keratinocyte culture, and consideration of candidate genes involved in epidermal growth regulationAntisense RNA-mediated deficiency of the calpain protease, nCL-4, in NIH3T3 cells is associated with neoplastic transformation and tumorigenesis.Gene expression profiling in limb-girdle muscular dystrophy 2ATherapeutic targeting of signaling pathways in muscular dystrophy.Alteration of sarcoplasmic reticulum ca release in skeletal muscle from calpain 3-deficient mice.Control of muscle fibre size: a crucial factor in ageing.Calpain inhibition protects against virus-induced apoptotic myocardial injury.The PEST domain of IkappaBalpha is necessary and sufficient for in vitro degradation by mu-calpain.Direct cleavage by the calcium-activated protease calpain can lead to inactivation of caspases.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.Calpain 3 is important for muscle regeneration: evidence from patients with limb girdle muscular dystrophies.Altered cross-bridge properties in skeletal muscle dystrophiesNuclear factor-kappa B signaling in skeletal muscle atrophy.Over-expression of BCL2 rescues muscle weakness in a mouse model of oculopharyngeal muscular dystrophy.Advances in the understanding of familial Mediterranean fever and possibilities for targeted therapy.Drug repositioning for orphan genetic diseases through Conserved Anticoexpressed Gene Clusters (CAGCs)Regulation of the M-cadherin-beta-catenin complex by calpain 3 during terminal stages of myogenic differentiationPathogenity of some limb girdle muscular dystrophy mutations can result from reduced anchorage to myofibrils and altered stability of calpain 3.Life or death by NFκB, Losartan promotes survival in dy2J/dy2J mouse of MDC1A.The genetic and molecular bases of monogenic disorders affecting proteolytic systems.Extensive scanning of the calpain-3 gene broadens the spectrum of LGMD2A phenotypes.Calpain-3 impairs cell proliferation and stimulates oxidative stress-mediated cell death in melanoma cells.Interplay of IKK/NF-kappaB signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy.Limb-girdle muscular dystrophies--from genetics to molecular pathology.
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P2860
Calpain 3 deficiency is associated with myonuclear apoptosis and profound perturbation of the IκBα/NF-κB pathway in limb-girdle muscular dystrophy type 2A
description
im Mai 1999 veröffentlichter wissenschaftlicher Artikel
@de
scientific article published on 01 May 1999
@en
wetenschappelijk artikel
@nl
наукова стаття, опублікована в травні 1999
@uk
name
Calpain 3 deficiency is associ ...... dle muscular dystrophy type 2A
@en
Calpain 3 deficiency is associ ...... dle muscular dystrophy type 2A
@nl
type
label
Calpain 3 deficiency is associ ...... dle muscular dystrophy type 2A
@en
Calpain 3 deficiency is associ ...... dle muscular dystrophy type 2A
@nl
prefLabel
Calpain 3 deficiency is associ ...... dle muscular dystrophy type 2A
@en
Calpain 3 deficiency is associ ...... dle muscular dystrophy type 2A
@nl
P2093
P50
P356
P1433
P1476
Calpain 3 deficiency is associ ...... dle muscular dystrophy type 2A
@en
P2093
J Loiselet
L V Anderson
P2888
P304
P356
10.1038/8385
P407
P577
1999-05-01T00:00:00Z