Myofiber integrity depends on desmin network targeting to Z-disks and costameres via distinct plectin isoforms. - Wikidata - awgldk - TriplyDB

Myofiber integrity depends on desmin network targeting to Z-disks and costameres via distinct plectin isoforms.

Myofiber integrity depends on desmin network targeting to Z-disks and costameres via distinct plectin isoforms.

http://www.wikidata.org/entity/Q36660691

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Mutation in exon 1f of PLEC, leading to disruption of plectin isoform 1f, causes autosomal-recessive limb-girdle muscular dystrophy Plectin isoform 1-dependent nuclear docking of desmin networks affects myonuclear architecture and expression of mechanotransducers.Targeted proteolysis of plectin isoform 1a accounts for hemidesmosome dysfunction in mice mimicking the dominant skin blistering disease EBS-Ogna The many faces of plectin and plectinopathies: pathology and mechanisms Hearts of dystonia musculorum mice display normal morphological and histological features but show signs of cardiac stress The Bin/amphiphysin/Rvs (BAR) domain protein endophilin B2 interacts with plectin and controls perinuclear cytoskeletal architecture.Intermediate filament-associated cytolinker plectin 1c destabilizes microtubules in keratinocytes The constant beat: cardiomyocytes adapt their forces by equal contraction upon environmental stiffening The specificity of the interaction between αB-crystallin and desmin filaments and its impact on filament aggregation and cell viability.Nebulin binding impedes mutant desmin filament assembly.Chemical chaperone ameliorates pathological protein aggregation in plectin-deficient muscle.Neuromuscular synapse integrity requires linkage of acetylcholine receptors to postsynaptic intermediate filament networks via rapsyn-plectin 1f complexes.Nebulette is a powerful cytolinker organizing desmin and actin in mouse hearts Reduced myofibrillar connectivity and increased Z-disk width in nebulin-deficient skeletal muscle.Detyrosinated microtubules buckle and bear load in contracting cardiomyocytes Muscle structure influences utrophin expression in mdx mice.Determining the mechanical properties of plectin in mouse myoblasts and keratinocytes.Myasthenic syndrome caused by plectinopathy The integrin-adhesome is required to maintain muscle structure, mitochondrial ATP production, and movement forces in Caenorhabditis elegans.Synemin isoforms differentially organize cell junctions and desmin filaments in neonatal cardiomyocytes Current status of the congenital myasthenic syndromes.Plectin isoform P1b and P1d deficiencies differentially affect mitochondrial morphology and function in skeletal muscle.Desminopathies: pathology and mechanisms.Cytoskeleton and regulation of mitochondrial function: the role of beta-tubulin II Downstream effects of plectin mutations in epidermolysis bullosa simplex with muscular dystrophy Linking cytoarchitecture to metabolism: sarcolemma-associated plectin affects glucose uptake by destabilizing microtubule networks in mdx myofibers.Plectin contributes to mechanical properties of living cells.Phosphorylation of serine 4,642 in the C-terminus of plectin by MNK2 and PKA modulates its interaction with intermediate filaments.Targeted inactivation of a developmentally regulated neural plectin isoform (plectin 1c) in mice leads to reduced motor nerve conduction velocity Plectin isoforms as organizers of intermediate filament cytoarchitecture.Plectin-intermediate filament partnership in skin, skeletal muscle, and peripheral nerve.Posttranslational modifications of desmin and their implication in biological processes and pathologies.The role of tubulin in the mitochondrial metabolism and arrangement in muscle cells.Desmin in muscle and associated diseases: beyond the structural function.Intermediate Filaments and the Plasma Membrane.Overview of the Muscle Cytoskeleton.Plectin isoform-dependent regulation of keratin-integrin alpha6beta4 anchorage via Ca2+/calmodulin Costamere remodeling with muscle loading and unloading in healthy young men.Stabilization of the dystroglycan complex in Cajal bands of myelinating Schwann cells through plectin-mediated anchorage to vimentin filaments.Type III Intermediate Filaments Desmin, Glial Fibrillary Acidic Protein (GFAP), Vimentin, and Peripherin.

P2860

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P2860

Myofiber integrity depends on desmin network targeting to Z-disks and costameres via distinct plectin isoforms.

http://www.wikidata.org/entity/Q36660691

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2008 nî lūn-bûn

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P2093

Anikó Zeöld

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Denise Paulin

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Gerhard Wiche

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Irmgard Fischer

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Rolf Schröder

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P2860

The interaction of dystrophin with beta-dystroglycan is regulated by tyrosine phosphorylation

Human synemin gene generates splice variants encoding two distinct intermediate filament proteins

Desmosomal dysfunction due to mutations in desmoplakin causes arrhythmogenic right ventricular dysplasia/cardiomyopathy

A missense mutation in the alphaB-crystallin chaperone gene causes a desmin-related myopathy

A dysfunctional desmin mutation in a patient with severe generalized myopathy

Missense mutations in desmin associated with familial cardiac and skeletal myopathy

Defective expression of plectin/HD1 in epidermolysis bullosa simplex with muscular dystrophy

A compound heterozygous one amino-acid insertion/nonsense mutation in the plectin gene causes epidermolysis bullosa simplex with plectin deficiency

Plectin is a linker of intermediate filaments to Z-discs in skeletal muscle fibers

Adhesion-dependent tyrosine phosphorylation of (beta)-dystroglycan regulates its interaction with utrophin

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4

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181

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Siegfried Reipert

Wolfram S. Kunz

Patryk Konieczny

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5358951

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18490514

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2386106

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