Pathophysiology of limb girdle muscular dystrophy type 2A: hypothesis and new insights into the IκBα/NF-κB survival pathway in skeletal muscle

No abstract is available for this paper.

• Publication year

  2001

• Venue

  Journal of molecular medicine

• Publication date

  2001-05-03

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1007/s001090100225
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar

• No claims are published for this paper.

• No concepts are published for this paper.

• The limb-girdle muscular dystrophies

  2003cited by this paper
• Secondary calpain3 deficiency in 2q-linked muscular dystrophy

  2001cited by this paper
• The Inhibition of Apoptosis in Myositis and in Normal Muscle Cells1

  2000influential reference
• DNA fragmentation occurs in skeletal muscle during tumor growth: A link with cancer cachexia?

  2000cited by this paper
• NF-kappaB-induced loss of MyoD messenger RNA: possible role in muscle decay and cachexia.

  2000cited by this paper
• Expression of human IAP-like protein in skeletal muscle: a possible explanation for the rare incidence of muscle fiber apoptosis in T-cell mediated inflammatory myopathies.

  2000cited by this paper
• Cell death in denervated skeletal muscle is distinct from classical apoptosis

  2000cited by this paper
• Identification and Characterization of Differentially Expressed Genes in Denervated Muscle

  2000cited by this paper
• Expression Profiling in the Muscular Dystrophies Identification of Novel Aspects of Molecular Pathophysiology

  2000cited by this paper
• Loss of Calpain 3 Proteolytic Activity Leads to Muscular Dystrophy and to Apoptosis-Associated Iκbα/Nuclear Factor κb Pathway Perturbation in Mice

  2000cited by this paper
• Calpain-3 gene expression is decreased during experimental cancer cachexia.

  2000cited by this paper
• Nuclear factor-κB regulates induction of apoptosis and inhibitor of apoptosis protein-1 expression in vascular smooth muscle cells

  1999cited by this paper
• Calpain 3 deficiency is associated with myonuclear apoptosis and profound perturbation of the IκBα/NF-κB pathway in limb-girdle muscular dystrophy type 2A

  1999cited by this paper
• Tumor Necrosis Factor-α-inducible IκBα Proteolysis Mediated by Cytosolic m-Calpain

  1999cited by this paper
• Nuclear factor-kappa B regulates induction of apoptosis and inhibitor of apoptosis protein-1 expression in vascular smooth muscle cells.

  1999influential reference
• Immunohistochemical analysis of Fas ligand expression in normal human tissues

  1999cited by this paper
• Localization of survival motor neuron protein in human apoptotic-like and regenerating muscle fibers, and neuromuscular junctions.

  1999cited by this paper
• IEX-1L, an Apoptosis Inhibitor Involved in NF-κB-Mediated Cell Survival

  1998cited by this paper
• ARC, an inhibitor of apoptosis expressed in skeletal muscle and heart that interacts selectively with caspases.

  1998cited by this paper
• FLIP prevents apoptosis induced by death receptors but not by perforin/granzyme B, chemotherapeutic drugs, and gamma irradiation.

  1998cited by this paper
• TRAF2 expression in differentiated muscle

  1998influential reference
• Possible Involvement of Fas-Mediated Apoptosis in Eye Muscle Tissue from Patients with Thyroid-Associated Ophthalmopathy

  1998cited by this paper
• Assignment of the tibial muscular dystrophy locus to chromosome 2q31.

  1998cited by this paper
• NF-kappa B and Rel proteins: evolutionarily conserved mediators of immune responses.

  1998cited by this paper
• Apoptotic myonuclei in human Duchenne muscular dystrophy.

  1998cited by this paper
• NF-kappaB antiapoptosis: induction of TRAF1 and TRAF2 and c-IAP1 and c-IAP2 to suppress caspase-8 activation.

  1998influential reference
• Functional Defects of a Muscle-specific Calpain, p94, Caused by Mutations Associated with Limb-Girdle Muscular Dystrophy Type 2A*

  1998cited by this paper
• IκB Is a Substrate for a Selective Pathway of Lysosomal Proteolysis

  1998cited by this paper
• DNA‐fragmentation and expression of apoptosis‐related proteins in experimentally denervated and reinnervated rat facial muscle

  1997cited by this paper
• Expression of Fas antigen is not associated with apoptosis in human myopathies

  1997cited by this paper
• Cytotoxic mechanisms in inflammatory myopathies. Co-expression of Fas and protective Bcl-2 in muscle fibres and inflammatory cells.

  1997cited by this paper
• Inhibition of death receptor signals by cellular FLIP

  1997cited by this paper
• Calpain Contributes to Silica-Induced IκB-α Degradation and Nuclear Factor-κB Activation

  1997cited by this paper
• Nuclear localization of I kappa B alpha promotes active transport of NF-kappa B from the nucleus to the cytoplasm.

  1997cited by this paper
• DNA‐fragmentation and apoptosis‐related proteins of muscle cells in motor neuron disorders

  1997cited by this paper
• Apoptosis‐related Proteins in Skeletal Muscle Fibers of Spinal Muscular Atrophy

  1997cited by this paper
• Structure and physiological function of calpains.

  1997cited by this paper
• Early lethality, functional NF-kappaB activation, and increased sensitivity to TNF-induced cell death in TRAF2-deficient mice.

  1997cited by this paper
• Calpain contributes to silica-induced I kappa B-alpha degradation and nuclear factor-kappa B activation.

  1997cited by this paper
• Myonuclear apoptosis in dystrophic mdx muscle occurs by perforin-mediated cytotoxicity.

  1997cited by this paper
• Muscle apoptosis in humans occurs in normal and denervated muscle, but not in myotonic dystrophy, dystrophinopathies or inflammatory disease

  1997cited by this paper
• THE NF-κB AND IκB PROTEINS: New Discoveries and Insights

  1996cited by this paper
• Juvenile limb-girdle muscular dystrophy. Clinical, histopathological and genetic data from a small community living in the Reunion Island.

  1996cited by this paper
• Natural Killer Cells and Macrophages in Immature Denervated Rat Muscles

  1996cited by this paper
• Localization of tumor necrosis factor-alpha and its receptors in inflammatory myopathies

  1996cited by this paper
• Signal-induced degradation of I kappa B alpha requires site-specific ubiquitination.

  1995cited by this paper
• Mutations in the proteolytic enzyme calpain 3 cause limb-girdle muscular dystrophy type 2A.

  1995cited by this paper
• Stimulation-dependent I kappa B alpha phosphorylation marks the NF-kappa B inhibitor for degradation via the ubiquitin-proteasome pathway.

  1995cited by this paper
• Inducible nuclear expression of newly synthesized I kappa B alpha negatively regulates DNA-binding and transcriptional activities of NF-kappa B

  1995cited by this paper
• Type II muscle fibers are stained by anti-Fas antibody.

  1995cited by this paper
• Distinct kinetics of subunit autolysis in mammalian m‐calpain activation

  1994cited by this paper
• Muscle-specific calpain, p94, is degraded by autolysis immediately after translation, resulting in disappearance from muscle.

  1993cited by this paper
• A gene for limb-girdle muscular dystrophy maps to chromosome 15 by linkage.

  1991cited by this paper
• Muscular dystrophy with separate clinical phenotypes in a large family

  1991cited by this paper
• Molecular cloning of a novel mammalian calcium-dependent protease distinct from both m- and mu-types. Specific expression of the mRNA in skeletal muscle.

  1989cited by this paper

• Alternations of NF-κB Signaling by Natural Compounds in Muscle-Derived Cancers

  2023cites this paper
• Integrating Untargeted and Targeted Metabolomics Coupled with Pathway Analysis Reveals Muscle Disorder in Osteoporosis on Orchiectomized Mice

  2023cites this paper
• Nuclei isolation methods fail to accurately assess the subcellular localization and behaviour of proteins in skeletal muscle

  2021cites this paper
• Limb-Girdle Muscular Dystrophy Type 2A (Leiden- Möbius Phenotype) with Compound Heterozygous Mutationin 4 Exon of CAPN3 Gene: Own Observation

  2020cites this paper
• Dietary myo‐inositol deficiency decreased intestinal immune function related to NF‐&kgr;B and TOR signaling in the intestine of young grass carp (Ctenopharyngodon idella)

  2018cites this paper
• Frzb gene expression regulation in vitro to restore muscle fibre homeostasis in limb-girdle muscular dystrophy type 2a (lgmd2a) and frzb-/- murine model muscle analysis

  2018cites this paper
• Optimal dietary protein level improved growth, disease resistance, intestinal immune and physical barrier function of young grass carp (Ctenopharyngodon idella).

  2016cites this paper
• Limb-girdle Muscular Dystrophies

  2015cites this paper
• Life or death by NFκB, Losartan promotes survival in dy2J/dy2J mouse of MDC1A

  2015cites this paper
• Involvement of oxidative stress, nuclear factor kappa B and the ubiquitin proteasomal pathway in dysferlinopathy.

  2014cites this paper
• The role of Calpain 3 in sheep skeletal muscle growth and post-mortem meat quality

  2014influential citation
• Oxidative Stress, NF-κB and the Ubiquitin Proteasomal Pathway in the Pathology of Calpainopathy

  2013cites this paper
• Skeletal muscle degenerative diseases and strategies for therapeutic muscle repair.

  2013cites this paper
• Aplicación de técnicas de proteómica para el estudio de enfermedades neuromusculares

  2012cites this paper
• Muscle Fiber Atrophy and Regeneration Coexist in Collagen VI–Deficient Human Muscle: Role of Calpain-3 and Nuclear Factor-&kgr;B Signaling

  2012cites this paper
• AAV-based shRNA silencing of NF-κB ameliorates muscle pathologies in mdx mice

  2012cites this paper
• The role of calpain in skeletal muscle

  2012cites this paper
• Muscle ankyrin repeat proteins: their role in striated muscle function in health and disease

  2011cites this paper
• Activation of skeletal muscle calpain-3 by eccentric exercise in humans does not result in its translocation to the nucleus or cytosol.

  2011cites this paper
• Calpain 3 Is a Rapid-Action, Unidirectional Proteolytic Switch Central to Muscle Remodeling

  2010cites this paper
• Differential gene expression profiling in iprobenfos-exposed marine medaka by heterologous microarray hybridization

  2010cites this paper
• Inhibition of the IKK/NF-κB pathway by AAV gene transfer improves muscle regeneration in older mdx mice

  2010cites this paper
• A new pathway encompassing calpain 3 and its newly identified substrate cardiac ankyrin repeat protein is involved in the regulation of the nuclear factor‐κB pathway in skeletal muscle

  2010cites this paper
• Proceedings of the Australian Physiological Society Symposium: Signals Mediating Exercise-Induced Skeletal Muscle Remodelling Calpains, skeletal muscle function and exercise

  2010cites this paper
• Endogenous Calpain-3 Activation Is Primarily Governed by Small Increases in Resting Cytoplasmic [Ca2+] and Is Not Dependent on Stretch*

  2009cites this paper
• Idiopathic inflammatory myopathies and the classical NF-kappaB complex: current insights and implications for therapy.

  2009cites this paper
• Dietary curcumin significantly improves obesity-associated inflammation and diabetes in mouse models of diabesity.

  2008cites this paper
• NF‐NF‐κBB‐dependent expression of the antiapoptotic factor c‐FLIP is regulated by calpain 3, the protein involved in limb‐girdle muscular dystrophy type 2A

  2008cites this paper
• NF-κB signaling in skeletal muscle: prospects for intervention in muscle diseases

  2008cites this paper
• Gene Expression Profiling in Limb-Girdle Muscular Dystrophy 2A

  2008cites this paper
• Bortezomib-Resistant Nuclear Factor-κB Activity in Multiple Myeloma Cells

  2008cites this paper
• Gene expression profile in iprobenfos exposed Medaka Fish by Microarray analysis

  2008cites this paper
• The Biology of Cancer Cachexia and the Role of TNF-α

  2007cites this paper
• Effect of anabolic agents on calpastatin promoters in porcine skeletal muscle and their responsiveness to cyclic adenosine monophosphate- and calcium-related stimuli.

  2006cites this paper
• 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 NFκB*

  2006cites this paper
• CAPN3 mutations in patients with idiopathic eosinophilic myositis

  2006cites this paper
• The childhood limb-girdle muscular dystrophies.

  2006cites this paper
• Limb girdle muscular dystrophy in the Hutterite population of North America

  2005cites this paper
• Muscle‐fiber apoptosis in neuromuscular diseases

  2005cites this paper
• The RAGE pathway in inflammatory myopathies and limb girdle muscular dystrophy

  2005cites this paper
• Nuclear Calpain Regulates Ca2+-dependent Signaling via Proteolysis of Nuclear Ca2+/Calmodulin-dependent Protein Kinase Type IV in Cultured Neurons*

  2005cites this paper
• Regulation of skeletal muscle proteolysis.

  2005cites this paper
• LGMD2A: genotype-phenotype correlations based on a large mutational survey on the calpain 3 gene.

  2005cites this paper
• Structure, régulation, expression et polymorphismes du gène CAST codant la calpastatine bovine

  2005influential citation
• Signaling pathways weigh in on decisions to make or break skeletal muscle

  2004cites this paper
• DIII Domain of Calpain 10 and Cpl Towards an Understanding of Calpain 10 Function

  2004cites this paper
• Calpain-related diseases.

  2004cites this paper
• A Common Haplotype Associated with the Basque 2362AG-TCATCT Mutation in the Muscular Calpain-3 Gene

  2004cites this paper
• Disuse atrophy and exercise rehabilitation in humans profoundly affects the expression of genes associated with the regulation of skeletal muscle mass

  2004cites this paper
• Nuclear factor-κB: its role in health and disease

  2004cites this paper
• Calpain 3 is expressed in astrocytes of rat and Microcebus brain.

  2003cites this paper
• Muscular dystrophies: genes to pathogenesis.

  2003cites this paper
• Activation of nuclear factor-κB in inflammatory myopathies and Duchenne muscular dystrophy

  2003cites this paper
• MOLECULAR GENETICS OF TIBIAL MUSCULAR DYSTROPHY (TMD) AND A NOVEL DISTAL MYOPATHY

  2003cites this paper
• Limb-girdle muscular dystrophy

  2003cites this paper
• Muscular dystrophy overview: genetics and diagnosis.

  2003cites this paper
• Activators and Target Genes of Rel/NF-кB Transcription Factors: From Bench to Bedside

  2003cites this paper
• Role of Apoptosis in Myopathies

  2003cites this paper
• Down-regulation of genes in the lysosomal and ubiquitin-proteasome proteolytic pathways in calpain-3-deficient muscle.

  2003cites this paper
• Asymptomatic carriers for homozygous novel mutations in the FKRP gene: the other end of the spectrum

  2003cites this paper
• Activation of an alternative NF‐ΚB pathway in skeletal muscle during disuse atrophy

  2002cites this paper