A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.
about
Identification of Xin-repeat proteins as novel ligands of the SH3 domains of nebulin and nebulette and analysis of their interaction during myofibril formation and remodelingThe Nebulin family: an actin support groupNebulin binding impedes mutant desmin filament assembly.Nebulette is a powerful cytolinker organizing desmin and actin in mouse heartsReduced myofibrillar connectivity and increased Z-disk width in nebulin-deficient skeletal muscle.αB-crystallin is a sensor for assembly intermediates and for the subunit topology of desmin intermediate filaments.Nebulin regulates actin filament lengths by a stabilization mechanism.New insights into the structural roles of nebulin in skeletal muscle.Nebulin, a major player in muscle health and diseaseCytoplasmic gamma-actin and tropomodulin isoforms link to the sarcoplasmic reticulum in skeletal muscle fibers.Increased desmin expression in hindlimb muscles of aging ratsThe desmin coil 1B mutation K190A impairs nebulin Z-disc assembly and destabilizes actin thin filaments.The sarcomeric protein nebulin: another multifunctional giant in charge of muscle strength optimizationNebulette knockout mice have normal cardiac function, but show Z-line widening and up-regulation of cardiac stress markers.Ubiquitylation by Trim32 causes coupled loss of desmin, Z-bands, and thin filaments in muscle atrophyTragedy in a heartbeat: malfunctioning desmin causes skeletal and cardiac muscle diseaseIntermediate filaments: primary determinants of cell architecture and plasticity.Lifting the nebula: novel insights into skeletal muscle contractility.Disorder profile of nebulin encodes a vernierlike position sensor for the sliding thin and thick filaments of the skeletal muscle sarcomere.Myofibril breakdown during atrophy is a delayed response requiring the transcription factor PAX4 and desmin depolymerization.HSPB7 is indispensable for heart development by modulating actin filament assembly.Molecular insights into cardiomyopathies associated with desmin (DES) mutations
P2860
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P2860
A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年学术文章
@wuu
2008年学术文章
@zh-cn
2008年学术文章
@zh-hans
2008年学术文章
@zh-my
2008年学术文章
@zh-sg
2008年學術文章
@yue
2008年學術文章
@zh
2008年學術文章
@zh-hant
name
A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.
@en
A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.
@nl
type
label
A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.
@en
A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.
@nl
prefLabel
A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.
@en
A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.
@nl
P2093
P2860
P356
P1476
A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.
@en
P2093
Carol C Gregorio
Gloria M Conover
Syerra N Henderson
P2860
P304
P356
10.1091/MBC.E08-07-0753
P577
2008-11-12T00:00:00Z