Recognising the potential of large animals for modelling neuromuscular junction physiology and disease.

NMDs NMJ disorders large animals peripheral neuropathy

Journal

Journal of anatomy
ISSN: 1469-7580
Titre abrégé: J Anat
Pays: England
ID NLM: 0137162

Informations de publication

Date de publication:
11 2022
Historique:
revised: 05 08 2022
received: 24 05 2022
accepted: 08 08 2022
pubmed: 4 9 2022
medline: 15 10 2022
entrez: 3 9 2022
Statut: ppublish

Résumé

The aetiology and pathophysiology of many diseases of the motor unit remain poorly understood and the role of the neuromuscular junction (NMJ) in this group of disorders is particularly overlooked, especially in humans, when these diseases are comparatively rare. However, elucidating the development, function and degeneration of the NMJ is essential to uncover its contribution to neuromuscular disorders, and to explore potential therapeutic avenues to treat these devastating diseases. Until now, an understanding of the role of the NMJ in disease pathogenesis has been hindered by inherent differences between rodent and human NMJs: stark contrasts in body size and corresponding differences in associated axon length underpin some of the translational issues in animal models of neuromuscular disease. Comparative studies in large mammalian models, including examination of naturally occurring, highly prevalent animal diseases and evaluation of their treatment, might provide more relevant insights into the pathogenesis and therapy of equivalent human diseases. This review argues that large animal models offer great potential to enhance our understanding of the neuromuscular system in health and disease, and in particular, when dealing with diseases for which nerve length dependency might underly the pathogenesis.

Identifiants

pubmed: 36056593
doi: 10.1111/joa.13749
pmc: PMC9558152
doi:

Types de publication

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

Langues

eng

Sous-ensembles de citation

IM

Pagination

1120-1132

Subventions

Organisme : Horserace Betting Levy Board
ID : vet/prj/780

Informations de copyright

© 2022 The Authors. Journal of Anatomy published by John Wiley & Sons Ltd on behalf of Anatomical Society.

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Auteurs

Stephen D Cahalan (SD)

Comparative Neuromuscular Diseases Laboratory, Department of Clinical Science and Services, Royal Veterinary College, University of London, London, UK.

Ines Boehm (I)

Edinburgh Medical School: Biomedical Sciences, University of Edinburgh, Edinburgh, UK.
Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh, UK.
Biozentrum University of Basel, Basel, Switzerland.

Ross A Jones (RA)

Edinburgh Medical School: Biomedical Sciences, University of Edinburgh, Edinburgh, UK.
Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh, UK.

Richard J Piercy (RJ)

Comparative Neuromuscular Diseases Laboratory, Department of Clinical Science and Services, Royal Veterinary College, University of London, London, UK.

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