Evidence map›Paper›PMID 27283349›Full record

ArticleScientific reports2016

Dynein disruption perturbs post-synaptic components and contributes to impaired MuSK clustering at the NMJ: implication in ALS.

Valérie Vilmont, Bruno Cadot, Elsa Vezin, Fabien Le Grand, Edgar R Gomes

Abstract read
In one paragraph

Article in Scientific reports, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.

0numbers the graph read from it
0cells of the map it votes in
15citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

15 citing papers in PubMed.

  1. Article
  2. Article
  3. Article
  4. Article
  5. Article
  6. Coexistence of myasthenia gravis and amyotrophic lateral sclerosis in a Bosnian male: an unusual clinical presentation.Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology · 2021
    Article
  7. Article
  8. Review
  9. Article
  10. Amyotrophic lateral sclerosis as a synaptopathy.Neural regeneration research · 2019
    Review
  11. miR126-5p Downregulation Facilitates Axon Degeneration and NMJ Disruption via a Non-Cell-Autonomous Mechanism in ALS.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2018
    Article
  12. Review
  13. Article
  14. EMBO molecular medicine · 2017
    Article
  15. In Vitro Differentiation of Mature Myofibers for Live Imaging.Journal of visualized experiments : JoVE · 2017
    Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

5 authors.

Valérie VilmontMyology Research Center, UM76-INSERM U974-CNRS FRE 3617 Sorbonne Universités, UPMC Université Paris 06, Paris, France.
Bruno CadotMyology Research Center, UM76-INSERM U974-CNRS FRE 3617 Sorbonne Universités, UPMC Université Paris 06, Paris, France.
Elsa VezinMyology Research Center, UM76-INSERM U974-CNRS FRE 3617 Sorbonne Universités, UPMC Université Paris 06, Paris, France.
Fabien Le GrandMyology Research Center, UM76-INSERM U974-CNRS FRE 3617 Sorbonne Universités, UPMC Université Paris 06, Paris, France.
Edgar R GomesMyology Research Center, UM76-INSERM U974-CNRS FRE 3617 Sorbonne Universités, UPMC Université Paris 06, Paris, France.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

The neuromuscular junction (NMJ) allows the transformation of a neuronal message into a mechanical force by muscle contraction and is the target of several neuromuscular disorders. While the neuronal side is under extensive research, the muscle appeared recently to have a growing role in the formation and integrity of the neuromuscular junction. We used an in vitro model of mature myofibers to study the role of dynein on major postsynaptic proteins. We found that dynein affects the expression and the clustering of acetylcholine receptors (AChRs), muscle specific tyrosine kinase (MuSK) and Rapsyn. We also show that myofibers with dynein impairment or from an amyotrophic lateral sclerosis (ALS) model (SOD1(G93A)) show similar defects in myofiber formation and agrin-induced AChR clustering suggesting a role for dynein impairment in ALS progression. Finally, we found that dynein can affect MuSK traffic through the endosomal pathway. Collectively, our studies show that defects in dynein can lead to impairment of muscle NMJ components' expression and clustering. We propose that NMJ defects could happen via defective MuSK traffic and that this could be one of the pathological features involved in neurodegeneration such as ALS.

Indexed as

AgrinAmyotrophic Lateral SclerosisAnimalsCell DifferentiationCells, CulturedDisease Models, AnimalDyneinsHumansMiceMice, TransgenicMuscle ProteinsMyoblastsNeuromuscular JunctionNocodazoleQuinazolinonesReceptor Protein-Tyrosine KinasesAgrinciliobrevin DDyneinsMuscle ProteinsMuSK protein, mouseNocodazoleperipheral membrane protein 43KQuinazolinonesReceptor Protein-Tyrosine KinasesReceptors, CholinergicRNA, Small InterferingSod1 protein, mouseSuperoxide Dismutase-1

Identifiers

PMID27283349
PMCPMC4901269

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.