Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
about
Cooperative control of striated muscle mass and metabolism by MuRF1 and MuRF2.Titin, a Central Mediator for Hypertrophic Signaling, Exercise-Induced Mechanosignaling and Skeletal Muscle RemodelingCongenital myopathies: an updateTropomodulin capping of actin filaments in striated muscle development and physiologySarcomeric pattern formation by actin cluster coalescenceIdentification of Xin-repeat proteins as novel ligands of the SH3 domains of nebulin and nebulette and analysis of their interaction during myofibril formation and remodelingPathophysiological concepts in the congenital myopathies: blurring the boundaries, sharpening the focusThe vertebrate muscle Z-disc: sarcomere anchor for structure and signallingThe Nebulin family: an actin support groupKLHL40 deficiency destabilizes thin filament proteins and promotes nemaline myopathyTropomodulin isoforms regulate thin filament pointed-end capping and skeletal muscle physiologyA nebulin ruler does not dictate thin filament lengthsLeiomodin-3-deficient mice display nemaline myopathy with fast-myofiber atrophyIdentification of 2 Potentially Relevant Gene Mutations Involved in Strabismus Using Whole-Exome SequencingThe nebulin SH3 domain is dispensable for normal skeletal muscle structure but is required for effective active load bearing in mouse.Leiomodin-3 dysfunction results in thin filament disorganization and nemaline myopathy.Neb: a zebrafish model of nemaline myopathy due to nebulin mutation.Visualization of cardiac muscle thin filaments and measurement of their lengths by electron tomographyClustered gene expression changes flank targeted gene loci in knockout mice.Loss of Smyhc1 or Hsp90alpha1 function results in different effects on myofibril organization in skeletal muscles of zebrafish embryosNebulin plays a direct role in promoting strong actin-myosin interactions.Reduced myofibrillar connectivity and increased Z-disk width in nebulin-deficient skeletal muscle.Differential splicing of the large sarcomeric protein nebulin during skeletal muscle developmentAltered myofilament function depresses force generation in patients with nebulin-based nemaline myopathy (NEM2).Nebulin regulates actin filament lengths by a stabilization mechanism.New insights into the structural roles of nebulin in skeletal muscle.Nebulin interactions with actin and tropomyosin are altered by disease-causing mutations.Changes in cross-bridge cycling underlie muscle weakness in patients with tropomyosin 3-based myopathy.Dynamic regulation of sarcomeric actin filaments in striated muscleJasplakinolide reduces actin and tropomyosin dynamics during myofibrillogenesisTroponin activator augments muscle force in nemaline myopathy patients with nebulin mutationsTissue triage and freezing for models of skeletal muscle disease.Nebulin deficiency in adult muscle causes sarcomere defects and muscle-type-dependent changes in trophicity: novel insights in nemaline myopathyNebulin, a major player in muscle health and diseaseFast skeletal muscle troponin activation increases force of mouse fast skeletal muscle and ameliorates weakness due to nebulin-deficiency.Loss of Tropomodulin4 in the zebrafish mutant träge causes cytoplasmic rod formation and muscle weakness reminiscent of nemaline myopathy.Early incorporation of obscurin into nascent sarcomeres: implication for myofibril assembly during cardiac myogenesisMuscle giants: molecular scaffolds in sarcomerogenesis.Leiomodin 3 and tropomodulin 4 have overlapping functions during skeletal myofibrillogenesis.Mutation update: the spectra of nebulin variants and associated myopathies.
P2860
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P2860
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
description
2006 nî lūn-bûn
@nan
2006 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
2006 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
2006年の論文
@ja
2006年論文
@yue
2006年論文
@zh-hant
2006年論文
@zh-hk
2006年論文
@zh-mo
2006年論文
@zh-tw
2006年论文
@wuu
name
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo
@nl
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
@ast
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
@en
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
@en-gb
type
label
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo
@nl
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
@ast
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
@en
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
@en-gb
prefLabel
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo
@nl
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
@ast
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
@en
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
@en-gb
P2093
P2860
P3181
P356
P1433
P1476
Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.
@en
P2093
Christian C Witt
Christoph Burkart
Dietmar Labeit
Henk Granzier
Mark McNabb
Siegfried Labeit
P2860
P304
P3181
P356
10.1038/SJ.EMBOJ.7601242
P407
P577
2006-08-10T00:00:00Z