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The toxic effect of R350P mutant desmin in striated muscle of man and mouseIntermediate Filaments Play a Pivotal Role in Regulating Cell Architecture and FunctionSignaling mechanisms and disrupted cytoskeleton in the diphenyl ditelluride neurotoxicityAAV9-mediated gene transfer of desmin ameliorates cardiomyopathy in desmin-deficient mice.Order and disorder in intermediate filament proteinsFBXO32, encoding a member of the SCF complex, is mutated in dilated cardiomyopathyN-acetyl-L-cysteine prevents stress-induced desmin aggregation in cellular models of desminopathyAntioxidant Treatment and Induction of Autophagy Cooperate to Reduce Desmin Aggregation in a Cellular Model of DesminopathyNebulin binding impedes mutant desmin filament assembly.Chemical chaperone ameliorates pathological protein aggregation in plectin-deficient muscle.Nebulette is a powerful cytolinker organizing desmin and actin in mouse heartsTargeted analysis of whole genome sequence data to diagnose genetic cardiomyopathy.Myofibrillar myopathies: new developments.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 desminopathyDeficiencies in tRNA synthetase editing activity cause cardioproteinopathy.Two desmin gene mutations associated with myofibrillar myopathies in Polish familiesAutophagic vacuolar pathology in desminopathies.Targeted sequencing and identification of genetic variants in sporadic inclusion body myositis.Abnormal accumulation of desmin in gastrocnemius myofibers of patients with peripheral artery disease: associations with altered myofiber morphology and density, mitochondrial dysfunction and impaired limb function.Protein post-translational modifications and misfolding: new concepts in heart failure.Exome sequencing identifies variants in two genes encoding the LIM-proteins NRAP and FHL1 in an Italian patient with BAG3 myofibrillar myopathy.Desmin modifications associate with amyloid-like oligomers deposition in heart failure.Genetics in dilated cardiomyopathy.Posttranslational modifications of desmin and their implication in biological processes and pathologies.Myofilament dysfunction as an emerging mechanism of volume overload heart failure.Desmosomes in the heart: a review of clinical and mechanistic analyses.Chaperoning heat shock proteins: proteomic analysis and relevance for normal and dystrophin-deficient muscle.Biomechanical characterization of myofibrillar myopathies.Desmin in muscle and associated diseases: beyond the structural function.Desmin related disease: a matter of cell survival failure.Mitochondrial abnormalities in the myofibrillar myopathies.The MOGE(S) classification : A TNM-like classification for cardiomyopathies.Mice lacking myosin IXb, an inflammatory bowel disease susceptibility gene, have impaired intestinal barrier function and superficial ulceration in the ileum.Desmin Mutation in the C-Terminal Domain Impairs Traction Force Generation in Myoblasts.Early signs of architectural and biomechanical failure in isolated myofibers and immortalized myoblasts from desmin-mutant knock-in miceAnimal Models of Congenital Cardiomyopathies Associated With Mutations in Z-Line Proteins.Intermediate Filaments: Structure and Assembly.Overview of the Muscle Cytoskeleton.
P2860
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P2860
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
Desminopathies: pathology and mechanisms.
@ast
Desminopathies: pathology and mechanisms.
@en
type
label
Desminopathies: pathology and mechanisms.
@ast
Desminopathies: pathology and mechanisms.
@en
prefLabel
Desminopathies: pathology and mechanisms.
@ast
Desminopathies: pathology and mechanisms.
@en
P2093
P2860
P921
P1476
Desminopathies: pathology and mechanisms.
@en
P2093
Christoph S Clemen
Harald Herrmann
Rolf Schröder
Sergei V Strelkov
P2860
P2888
P356
10.1007/S00401-012-1057-6
P577
2012-11-11T00:00:00Z
2013-01-01T00:00:00Z