Unlocking the Complexity of Neuromuscular Diseases: Insights from Human Pluripotent Stem Cell-Derived Neuromuscular Junctions.

human neuromuscular junction in vitro models microfluidics and organoids pluripotent stem cells

Journal

International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791

Informations de publication

Date de publication:
18 Oct 2023
Historique:
received: 11 09 2023
revised: 26 09 2023
accepted: 16 10 2023
medline: 30 10 2023
pubmed: 28 10 2023
entrez: 28 10 2023
Statut: epublish

Résumé

Over the past 20 years, the use of pluripotent stem cells to mimic the complexities of the human neuromuscular junction has received much attention. Deciphering the key mechanisms underlying the establishment and maturation of this complex synapse has been driven by the dual goals of addressing developmental questions and gaining insight into neuromuscular disorders. This review aims to summarise the evolution and sophistication of in vitro neuromuscular junction models developed from the first differentiation of human embryonic stem cells into motor neurons to recent neuromuscular organoids. We also discuss the potential offered by these models to decipher different neuromuscular diseases characterised by defects in the presynaptic compartment, the neuromuscular junction, and the postsynaptic compartment. Finally, we discuss the emerging field that considers the use of these techniques in drug screening assay and the challenges they will face in the future.

Identifiants

pubmed: 37894969
pii: ijms242015291
doi: 10.3390/ijms242015291
pmc: PMC10607237
pii:
doi:

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

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Auteurs

Morgan Gazzola (M)

INSERM U861, Institute for Stem Cell Therapy and Exploration of Monogenic Diseases, 91100 Corbeil-Essonnes, France.

Cécile Martinat (C)

INSERM U861, Institute for Stem Cell Therapy and Exploration of Monogenic Diseases, 91100 Corbeil-Essonnes, France.

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