(Q35388987)

8 August 2017

title

The desmin coil 1B mutation K190A impairs nebulin Z-disc assembly and destabilizes actin thin filaments (English)

1 reference

8 August 2017

1 reference

1

Gloria M Conover

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8 August 2017

Carol C Gregorio

2

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8 August 2017

language of work or name

English

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publication date

7 October 2011

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8 August 2017

published in

Journal of Cell Science

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8 August 2017

volume

124

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8 August 2017

issue

Pt 20

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8 August 2017

page(s)

3464-3476

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describes a project that uses

8 August 2017

ImageJ

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC3196858/fullTextXML

inferred from PubMed Central ID database lookup

cites work

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Nebulin regulates actin filament lengths by a stabilization mechanism.

27 July 2018

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Reduced myofibrillar connectivity and increased Z-disk width in nebulin-deficient skeletal muscle

27 July 2018

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Altered myofilament function depresses force generation in patients with nebulin-based nemaline myopathy (NEM2).

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Nebulin alters cross-bridge cycling kinetics and increases thin filament activation: a novel mechanism for increasing tension and reducing tension cost

27 July 2018

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Nebulin plays a direct role in promoting strong actin-myosin interactions

27 July 2018

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Intermediate filaments exchange subunits along their length and elongate by end-to-end annealing

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Severe infantile-onset cardiomyopathy associated with a homozygous deletion in desmin

27 July 2018

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Reduced thin filament length in nebulin-knockout skeletal muscle alters isometric contractile properties

27 July 2018

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A nebulin ruler does not dictate thin filament lengths

27 July 2018

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How much mutant protein is needed to cause a protein aggregate myopathy in vivo? Lessons from an exceptional desminopathy

27 July 2018

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Nebulin interacts with CapZ and regulates thin filament architecture within the Z-disc

27 July 2018

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Intermediate filaments: versatile building blocks of cell structure

27 July 2018

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Proper perinuclear localization of the TRIM-like protein myospryn requires its binding partner desmin.

27 July 2018

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27 July 2018

Primary desminopathies

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Towards a molecular description of intermediate filament structure and assembly

27 July 2018

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

27 July 2018

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

27 July 2018

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27 July 2018

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Nebulin regulates the assembly and lengths of the thin filaments in striated muscle

27 July 2018

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

27 July 2018

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27 July 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Desmin cytoskeleton: a potential regulator of muscle mitochondrial behavior and function

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Sarcolemmal organization in skeletal muscle lacking desmin: evidence for cytokeratins associated with the membrane skeleton at costameres

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Each actin subunit has three nebulin binding sites: implications for steric blocking

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

The NH2 terminus of titin spans the Z-disc: its interaction with a novel 19-kD ligand (T-cap) is required for sarcomeric integrity

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

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

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Missense mutations in desmin associated with familial cardiac and skeletal myopathy

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Molecular structure of the sarcomeric Z-disk: two types of titin interactions lead to an asymmetrical sorting of alpha-actinin.

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Two-hybrid analysis reveals fundamental differences in direct interactions between desmoplakin and cell type-specific intermediate filaments

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Disruption of muscle architecture and myocardial degeneration in mice lacking desmin

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Human skeletal muscle nebulin sequence encodes a blueprint for thin filament architecture. Sequence motifs and affinity profiles of tandem repeats and terminal SH3.

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Cardiovascular lesions and skeletal myopathy in mice lacking desmin.

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

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

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

A network of transverse and longitudinal intermediate filaments is associated with sarcomeres of adult vertebrate skeletal muscle

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Specific attachment of desmin filaments to desmosomal plaques in cardiac myocytes

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Distributions of vimentin and desmin in developing chick myotubes in vivo. II. Immunoelectron microscopic study

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Binding of two desmin derivatives to the plasma membrane and the nuclear envelope of avian erythrocytes: evidence for a conserved site-specificity in intermediate filament-membrane interactions

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Effects of CapZ, an actin capping protein of muscle, on the polymerization of actin

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Evidence that nebulin is a protein-ruler in muscle thin filaments

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Nebulin as a length regulator of thin filaments of vertebrate skeletal muscles: correlation of thin filament length, nebulin size, and epitope profile

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Continuous growth of vimentin filaments in mouse fibroblasts

27 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

27 July 2018

Desmin-related myopathy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Theoretical predictions of the effects of force transmission by desmin on intersarcomere dynamics

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Desmin myopathy with severe cardiomyopathy in a Uruguayan family due to a codon deletion in a new location within the desmin 1A rod domain

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Muscle giants: molecular scaffolds in sarcomerogenesis

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Neurofilaments bind tubulin and modulate its polymerization

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Desmin and vimentin intermediate filament networks: their viscoelastic properties investigated by mechanical rheometry.

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

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

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

A quantitative kinetic model for the in vitro assembly of intermediate filaments from tetrameric vimentin.

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

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

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Desmin accumulation restrictive cardiomyopathy and atrioventricular block associated with desmin gene defects.

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Mapping and consensus sequence identification for multiple vinculin binding sites within the talin rod.

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

SPOT synthesis: reliability of array-based measurement of peptide binding affinity.

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Intermediate filament associated proteins

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

The complete mouse nebulin gene sequence and the identification of cardiac nebulin

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Molecular dissection of the interaction of desmin with the C-terminal region of nebulin

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Initiation and maturation of I-Z-I bodies in the growth tips of transfected myotubes.

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Titin and nebulin: protein rulers in muscle?

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Correlation Between Conformational and Binding Properties of Nebulin Repeats

26 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3196858

Mutations in desmin's carboxy-terminal "tail" domain severely modify filament and network mechanics

8 December 2018

1 reference

12 December 2020

A series of Chinese patients with desminopathy associated with six novel and one reported mutations in the desmin gene

1 reference

12 December 2020

Autophagy in desmin-related cardiomyopathy: thoughts at the halfway point

1 reference

12 December 2020

Expression of R120G-alphaB-crystallin causes aberrant desmin and alphaB-crystallin aggregation and cardiomyopathy in mice

1 reference

12 December 2020

PEVK Domain of Titin: An Entropic Spring with Actin-Binding Properties

1 reference

12 December 2020

Nebulin is a thin filament protein of the cardiac muscle of the agnathans

1 reference

12 December 2020

Evidence for a direct but sequential binding of titin to tropomyosin and actin filaments

1 reference

12 December 2020

Molecular analysis of intermediate filament cytoskeleton--a putative load-bearing structure

1 reference

12 December 2020

Nebulin as a giant actin-binding template protein in skeletal muscle sarcomere. Interaction of actin and cloned human nebulin fragments

1 reference

12 December 2020

Nebulin is a full-length template of actin filaments in the skeletal muscle sarcomere: an immunoelectron microscopic study of its orientation and span with site-specific monoclonal antibodies

1 reference

12 December 2020

Tissue-specific expression and alpha-actinin binding properties of the Z-disc titin: implications for the nature of vertebrate Z-discs

1 reference

12 December 2020

Distribution and organization of desmin in cultured adult cardiac muscle cells: reflection on function

1 reference

12 December 2020

Prevalence of Desmin Mutations in Dilated Cardiomyopathy

1 reference

12 December 2020

Identifiers

DOI

10.1242/JCS.087080

1 reference

8 August 2017

1 reference

8 August 2017

1 reference