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

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          Abstract

          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.

          Abstract

          The role of the neuromuscular junction in neuromuscular disorders is not fully understood. Naturally occurring neuromuscular diseases in large animals might offer translational benefits over lab rodent studies.

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          Most cited references118

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          Transcriptome-scale super-resolved imaging in tissues by RNA seqFISH+

          Imaging the transcriptome in situ with high accuracy has been a major challenge in single cell biology, particularly hindered by the limits of optical resolution and the density of transcripts in single cells 1–5 . Here, we demonstrate seqFISH+, that can image the mRNAs for 10,000 genes in single cells with high accuracy and sub-diffraction-limit resolution, in the mouse brain cortex, subventricular zone, and the olfactory bulb, using a standard confocal microscope. The transcriptome level profiling of seqFISH+ allows unbiased identification of cell classes and their spatial organization in tissues. In addition, seqFISH+ reveals subcellular mRNA localization patterns in cells and ligand-receptor pairs across neighboring cells. This technology demonstrates the ability to generate spatial cell atlases and to perform discovery-driven studies of biological processes in situ.
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            dendextend: an R package for visualizing, adjusting and comparing trees of hierarchical clustering

            Tal Galili (2015)
            Summary: dendextend is an R package for creating and comparing visually appealing tree diagrams. dendextend provides utility functions for manipulating dendrogram objects (their color, shape and content) as well as several advanced methods for comparing trees to one another (both statistically and visually). As such, dendextend offers a flexible framework for enhancing R's rich ecosystem of packages for performing hierarchical clustering of items. Availability and implementation: The dendextend R package (including detailed introductory vignettes) is available under the GPL-2 Open Source license and is freely available to download from CRAN at: (http://cran.r-project.org/package=dendextend) Contact: Tal.Galili@math.tau.ac.il
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              Stem cells: past, present, and future

              In recent years, stem cell therapy has become a very promising and advanced scientific research topic. The development of treatment methods has evoked great expectations. This paper is a review focused on the discovery of different stem cells and the potential therapies based on these cells. The genesis of stem cells is followed by laboratory steps of controlled stem cell culturing and derivation. Quality control and teratoma formation assays are important procedures in assessing the properties of the stem cells tested. Derivation methods and the utilization of culturing media are crucial to set proper environmental conditions for controlled differentiation. Among many types of stem tissue applications, the use of graphene scaffolds and the potential of extracellular vesicle-based therapies require attention due to their versatility. The review is summarized by challenges that stem cell therapy must overcome to be accepted worldwide. A wide variety of possibilities makes this cutting edge therapy a turning point in modern medicine, providing hope for untreatable diseases.
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                Author and article information

                Contributors
                rpiercy@rvc.ac.uk
                Journal
                J Anat
                J Anat
                10.1111/(ISSN)1469-7580
                JOA
                Journal of Anatomy
                John Wiley and Sons Inc. (Hoboken )
                0021-8782
                1469-7580
                02 September 2022
                November 2022
                02 September 2022
                : 241
                : 5 ( doiID: 10.1111/joa.v241.5 )
                : 1120-1132
                Affiliations
                [ 1 ] Comparative Neuromuscular Diseases Laboratory, Department of Clinical Science and Services, Royal Veterinary College University of London London UK
                [ 2 ] Edinburgh Medical School: Biomedical Sciences University of Edinburgh Edinburgh UK
                [ 3 ] Euan MacDonald Centre for Motor Neurone Disease Research University of Edinburgh Edinburgh UK
                [ 4 ] Biozentrum University of Basel Basel Switzerland
                Author notes
                [*] [* ] Correspondence

                Richard J. Piercy, Royal Veterinary College, University of London, Camden, London, NW1 0TU, UK.

                Email: rpiercy@ 123456rvc.ac.uk

                Author information
                https://orcid.org/0000-0003-2287-1416
                https://orcid.org/0000-0002-9343-0944
                https://orcid.org/0000-0002-4344-6438
                Article
                JOA13749 JANAT-2022-0184.R1
                10.1111/joa.13749
                9558152
                36056593
                35fccd09-8489-4db7-89f7-f48ed20f04b1
                © 2022 The Authors. Journal of Anatomy published by John Wiley & Sons Ltd on behalf of Anatomical Society.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                : 05 August 2022
                : 24 May 2022
                : 08 August 2022
                Page count
                Figures: 5, Tables: 0, Pages: 13, Words: 10043
                Funding
                Funded by: Anatomical Society , doi 10.13039/100012070;
                Award ID: PhD Studentship
                Funded by: Horserace Betting Levy Board , doi 10.13039/501100001280;
                Award ID: vet/prj/780
                Categories
                Review Article
                Review Article
                Custom metadata
                2.0
                November 2022
                Converter:WILEY_ML3GV2_TO_JATSPMC version:6.2.0 mode:remove_FC converted:13.10.2022

                Anatomy & Physiology
                large animals,nmds,nmj disorders,peripheral neuropathy
                Anatomy & Physiology
                large animals, nmds, nmj disorders, peripheral neuropathy

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