Expression of ALS-linked SOD1 Mutation in Motoneurons or Myotubes Induces Differential Effects on Neuromuscular Function In vitro.

Amyotrophic Lateral Sclerosis / genetics Animals Disease Models, Animal Mice Mice, Transgenic Motor Neurons Muscle Fibers, Skeletal Mutation Neurodegenerative Diseases Superoxide Dismutase / genetics Superoxide Dismutase-1 / genetics
amyotophic lateral sclerosis electrical activity mouse primary cell culture myotube contraction neuromuscular junction

Journal

Neuroscience
ISSN: 1873-7544
Titre abrégé: Neuroscience
Pays: United States
ID NLM: 7605074

Informations de publication

Date de publication:
21 05 2020
Historique:
received: 19 08 2019
revised: 25 03 2020
accepted: 26 03 2020
pubmed: 3 4 2020
medline: 15 5 2021
entrez: 3 4 2020
Statut: ppublish

Résumé

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that selectively affects upper and lower motoneurons. Dismantlement of the neuromuscular junction (NMJ) is an early pathological hallmark of the disease whose cellular origin remains still debated. We developed an in vitro NMJ model to investigate the differential contribution of motoneurons and muscle cells expressing ALS-causing mutation in the superoxide dismutase 1 (SOD1) to neuromuscular dysfunction. The primary co-culture system allows the formation of functional NMJs and fosters the expression of the ALS-sensitive fast fatigable type II-b myosin heavy chain (MHC) isoform. Expression of SOD1

Identifiants

DOI: 10.1016/j.neuroscience.2020.03.044 PMID: 32234507
pubmed: 32234507
pii: S0306-4522(20)30203-7
doi: 10.1016/j.neuroscience.2020.03.044
pii:
doi:

Substances chimiques

Sod1 protein, mouse EC 1.15.1.1
Superoxide Dismutase EC 1.15.1.1
Superoxide Dismutase-1 EC 1.15.1.1

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

33-43

Informations de copyright

Copyright © 2020 IBRO. Published by Elsevier Ltd. All rights reserved.

Auteurs

Salim Benlefki (S)

The Neuroscience Institute of Montpellier, Inserm UMR1051, Montpellier University, Saint Eloi Hospital, Montpellier, France.

Ana Sanchez-Vicente (A)

The Neuroscience Institute of Montpellier, Inserm UMR1051, Montpellier University, Saint Eloi Hospital, Montpellier, France.

Vanessa Milla (V)

The Neuroscience Institute of Montpellier, Inserm UMR1051, Montpellier University, Saint Eloi Hospital, Montpellier, France.

Olivier Lucas (O)

The Neuroscience Institute of Montpellier, Inserm UMR1051, Montpellier University, Saint Eloi Hospital, Montpellier, France.

Claire Soulard (C)

The Neuroscience Institute of Montpellier, Inserm UMR1051, Montpellier University, Saint Eloi Hospital, Montpellier, France.

Richard Younes (R)

The Neuroscience Institute of Montpellier, Inserm UMR1051, Montpellier University, Saint Eloi Hospital, Montpellier, France; Neuroscience Research Center, Faculty of Medical Sciences, Lebanese University, Beirut, Lebanon.

Csilla Gergely (C)

Charles Coulomb Laboratory, L2C, UMR5221, Montpellier University, CNRS, Montpellier, France.

Mélissa Bowerman (M)

The Neuroscience Institute of Montpellier, Inserm UMR1051, Montpellier University, Saint Eloi Hospital, Montpellier, France.

Cédric Raoul (C)

The Neuroscience Institute of Montpellier, Inserm UMR1051, Montpellier University, Saint Eloi Hospital, Montpellier, France.

Frédérique Scamps (F)

The Neuroscience Institute of Montpellier, Inserm UMR1051, Montpellier University, Saint Eloi Hospital, Montpellier, France. Electronic address: frederique.scamps@inserm.fr.

Cécile Hilaire (C)

The Neuroscience Institute of Montpellier, Inserm UMR1051, Montpellier University, Saint Eloi Hospital, Montpellier, France. Electronic address: cecile.hilaire@umontpellier.fr.

Articles similaires

Evaluating the efficacy of telesurgery with dual console SSI Mantra Surgical Robotic System: experiment on animal model and clinical trials.

Sudhir Prem Srivastava, Vishwajyoti Pascual Srivastava, Avinesh Singh et al.
1.00
Robotic Surgical Procedures Animals Humans Telemedicine Models, Animal

Odour generalisation and detection dog training.

Lyn Caldicott, Thomas W Pike, Helen E Zulch et al.
1.00
Animals Odorants Dogs Generalization, Psychological Smell

FBXO22 inhibits colitis and colorectal carcinogenesis by regulating the degradation of the S2448-phosphorylated form of mTOR.

Minle Li, Xuan Chen, Pengfei Qu et al.
1.00
Animals TOR Serine-Threonine Kinases Colorectal Neoplasms Colitis Mice

Use of organic material provided by an automatic enrichment device by weaner pigs and its influence on tail lesions.

Philipp Heseker, Jeanette Probst, Stefanie Ammer et al.
1.00
Animals Tail Swine Behavior, Animal Animal Husbandry

Classifications MeSH

questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Troubles de l'homéostasie des protéines › Protéinopathies TDP-43 › Sclérose latérale amyotrophique › Expression of ALS-linked SOD1 Mutation in...
questionsmedicales.fr › Eucaryotes › Animaux › Expression of ALS-linked SOD1 Mutation in...
questionsmedicales.fr › Techniques d'investigation › Modèles théoriques › Modèles biologiques › Modèles animaux de maladie humaine › Expression of ALS-linked SOD1 Mutation in...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › Expression of ALS-linked SOD1 Mutation in...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › Souris transgéniques › Expression of ALS-linked SOD1 Mutation in...
questionsmedicales.fr › Cellules › Neurones › Neurones efférents › Motoneurones › Expression of ALS-linked SOD1 Mutation in...
questionsmedicales.fr › Cellules › Cellules musculaires › Fibres musculaires squelettiques › Expression of ALS-linked SOD1 Mutation in...
questionsmedicales.fr › Phénomènes génétiques › Variation génétique › Mutation › Expression of ALS-linked SOD1 Mutation in...
questionsmedicales.fr › Maladies du système nerveux › Maladies neurodégénératives › Expression of ALS-linked SOD1 Mutation in...
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Oxidoreductases › Superoxide dismutase › Expression of ALS-linked SOD1 Mutation in...
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Oxidoreductases › Superoxide dismutase › Superoxide dismutase-1 › Expression of ALS-linked SOD1 Mutation in...