Severe muscle disease-causing desmin mutations interfere with in vitro filament assembly at distinct stages - Wikidata - awgldk - TriplyDB

Severe muscle disease-causing desmin mutations interfere with in vitro filament assembly at distinct stages

Severe muscle disease-causing desmin mutations interfere with in vitro filament assembly at distinct stages

http://www.wikidata.org/entity/Q34075709

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A cardiac myosin light chain kinase regulates sarcomere assembly in the vertebrate heart.The toxic effect of R350P mutant desmin in striated muscle of man and mouse Dual color photoactivation localization microscopy of cardiomyopathy-associated desmin mutants Intermediate Filaments Play a Pivotal Role in Regulating Cell Architecture and Function N-acetyl-L-cysteine prevents stress-induced desmin aggregation in cellular models of desminopathy Antioxidant Treatment and Induction of Autophagy Cooperate to Reduce Desmin Aggregation in a Cellular Model of Desminopathy Monitoring intermediate filament assembly by small-angle x-ray scattering reveals the molecular architecture of assembly intermediates.SUMO regulates the assembly and function of a cytoplasmic intermediate filament protein in C. elegans Nebulin binding impedes mutant desmin filament assembly.Nebulette is a powerful cytolinker organizing desmin and actin in mouse hearts Severe infantile-onset cardiomyopathy associated with a homozygous deletion in desmin.Tensile properties of single desmin intermediate filaments.αB-crystallin is a sensor for assembly intermediates and for the subunit topology of desmin intermediate filaments.Sequence-resolved free energy profiles of stress-bearing vimentin intermediate filaments.Differential proteomic analysis of abnormal intramyoplasmic aggregates in desminopathy Withaferin A effectively targets soluble vimentin in the glaucoma filtration surgical model of fibrosis.The Alexander disease-causing glial fibrillary acidic protein mutant, R416W, accumulates into Rosenthal fibers by a pathway that involves filament aggregation and the association of alpha B-crystallin and HSP27 The desmin coil 1B mutation K190A impairs nebulin Z-disc assembly and destabilizes actin thin filaments.Desminopathies: pathology and mechanisms.Myopathic lamin mutations impair nuclear stability in cells and tissue and disrupt nucleo-cytoskeletal coupling.Primary desminopathies A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.Characterizing the normal proteome of human ciliary body.Tragedy in a heartbeat: malfunctioning desmin causes skeletal and cardiac muscle disease Intermediate filaments: primary determinants of cell architecture and plasticity.Designing heart performance by gene transfer.Intermediate filament diseases: desminopathy.Biomechanical characterization of myofibrillar myopathies.Desmin related disease: a matter of cell survival failure.Desmin A213V substitution represents a rare polymorphism but not a mutation and is more prevalent in patients with heart dilation of various origins.Desmin Mutation in the C-Terminal Domain Impairs Traction Force Generation in Myoblasts.Intermediate Filaments: Structure and Assembly.The domain organization of the bacterial intermediate filament-like protein crescentin is important for assembly and function.A missense mutation in desmin tail domain linked to human dilated cardiomyopathy promotes cleavage of the head domain and abolishes its Z-disc localization.Intermediate filaments and cellular mechanics.A quantitative kinetic model for the in vitro assembly of intermediate filaments from tetrameric vimentin.Molecular insights into cardiomyopathies associated with desmin (DES) mutations

P2860

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P2860

Severe muscle disease-causing desmin mutations interfere with in vitro filament assembly at distinct stages

http://www.wikidata.org/entity/Q34075709

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2005 nî lūn-bûn

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2005 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած

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Proceedings of the National Academy of Sciences of the United States of America

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Severe muscle disease-causing ...... nt assembly at distinct stages

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Anna Kostareva

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Gunnar Sjöberg

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Harald Bär

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Harald Herrmann

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Norbert Mücke

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P2860

Conserved segments 1A and 2B of the intermediate filament dimer: their atomic structures and role in filament assembly

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

Immunological characterization of the subunit of the 100 A filaments from muscle cells

Intermediate filaments: molecular structure, assembly mechanism, and integration into functionally distinct intracellular Scaffolds

Plakins: goliaths that link cell junctions and the cytoskeleton

Myofibrillar myopathy: clinical, morphological and genetic studies in 63 patients

Acute effects of desmin mutations on cytoskeletal and cellular integrity in cardiac myocytes

Intermediate filaments: structure, dynamics, function, and disease

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