A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture. - Wikidata - wikimedia - TriplyDB

A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.

A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.

http://www.wikidata.org/entity/Q37078581

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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 remodeling The Nebulin family: an actin support group Nebulin binding impedes mutant desmin filament assembly.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.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 disease Cytoplasmic gamma-actin and tropomodulin isoforms link to the sarcoplasmic reticulum in skeletal muscle fibers.Increased desmin expression in hindlimb muscles of aging rats The 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 optimization Nebulette 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 atrophy Tragedy in a heartbeat: malfunctioning desmin causes skeletal and cardiac muscle disease Intermediate 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

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P2860

A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.

http://www.wikidata.org/entity/Q37078581

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

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MBC.E08-07-0753

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Molecular Biology of the Cell

P1476

A myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.

@en

P2093

Carol C Gregorio

http://www.w3.org/2001/XMLSchema#string

Gloria M Conover

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Syerra N Henderson

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P2860

Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.

Characterization of nebulette and nebulin and emerging concepts of their roles for vertebrate Z-discs

The complete primary structure of human nebulin and its correlation to muscle structure

Structural and functional roles of desmin in mouse skeletal muscle during passive deformation.

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

Desmin is essential for the tensile strength and integrity of myofibrils but not for myogenic commitment, differentiation, and fusion of skeletal muscle

Nebulin-deficient mice exhibit shorter thin filament lengths and reduced contractile function in skeletal muscle

A novel marker for vertebrate embryonic heart, the EH-myomesin isoform

M band proteins myomesin and skelemin are encoded by the same gene: analysis of its organization and expression

Striated muscle cytoarchitecture: an intricate web of form and function

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10.1091/MBC.E08-07-0753

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3

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