Comparative Neuromuscular Diseases Laboratory, Department of Clinical Science and Services, Royal Veterinary College, London, UK.
Edinburgh Medical School: Biomedical Sciences, University of Edinburgh, Edinburgh, UK.
J Anat. 2022 Nov;241(5):1133-1147. doi: 10.1111/joa.13747. Epub 2022 Sep 10.
Morphological study of the neuromuscular junction (NMJ), a specialised peripheral synapse formed between a lower motor neuron and skeletal muscle fibre, has significantly contributed to the understanding of synaptic biology and neuromuscular disease pathogenesis. Rodent NMJs are readily accessible, and research into conditions such as amyotrophic lateral sclerosis (ALS), Charcot-Marie-Tooth disease (CMT), and spinal muscular atrophy (SMA) has relied heavily on experimental work in these small mammals. However, given that nerve length dependency is an important feature of many peripheral neuropathies, these rodent models have clear shortcomings; large animal models might be preferable, but their size presents novel anatomical challenges. Overcoming these constraints to study the NMJ morphology of large mammalian distal limb muscles is of prime importance to increase cross-species translational neuromuscular research potential, particularly in the study of long motor units. In the past, NMJ phenotype analysis of large muscle bodies within the equine distal pelvic limb, such as the tibialis cranialis, or within muscles of high fibrous content, such as the soleus, has posed a distinct experimental hurdle. We optimised a technique for NMJ location and dissection from equine pelvic limb muscles. Using a quantification method validated in smaller species, we demonstrate their morphology and show that equine NMJs can be reliably dissected, stained and analysed. We reveal that the NMJs within the equine soleus have distinctly different morphologies when compared to the extensor digitorum longus and tibialis cranialis muscles. Overall, we demonstrate that equine distal pelvic limb muscles can be regionally dissected, with samples whole-mounted and their innervation patterns visualised. These methods will allow the localisation and analysis of neuromuscular junctions within the muscle bodies of large mammals to identify neuroanatomical and neuropathological features.
神经肌肉接头(NMJ)的形态学研究是一种在运动神经元和骨骼肌纤维之间形成的专门的外周突触,对突触生物学和神经肌肉疾病发病机制的理解有重要贡献。啮齿动物 NMJ 很容易接近,对肌萎缩侧索硬化症(ALS)、Charcot-Marie-Tooth 病(CMT)和脊髓性肌萎缩症(SMA)等疾病的研究在很大程度上依赖于这些小型哺乳动物的实验工作。然而,鉴于神经长度依赖性是许多周围神经病变的一个重要特征,这些啮齿动物模型有明显的缺陷;大型动物模型可能更可取,但它们的体型带来了新的解剖学挑战。克服这些限制,研究大型哺乳动物远肢肌肉的 NMJ 形态对于增加跨物种转化神经肌肉研究的潜力非常重要,特别是在长运动单位的研究中。过去,在马的远肢骨盆肌肉(如胫骨前肌)或高纤维含量的肌肉(如比目鱼肌)中,对大型肌肉体的 NMJ 表型分析一直是一个明显的实验障碍。我们优化了一种从马的骨盆肢肌肉中定位和分离 NMJ 的技术。我们使用在较小物种中验证的定量方法,展示了它们的形态,并表明可以可靠地分离、染色和分析马的 NMJ。我们发现,与伸趾长肌和胫骨前肌相比,马的比目鱼肌中的 NMJ 具有明显不同的形态。总的来说,我们证明了马的远肢骨盆肌肉可以进行区域性解剖,将样本整体安装并可视化其神经支配模式。这些方法将允许在大型哺乳动物的肌肉体内定位和分析神经肌肉接头,以识别神经解剖和神经病理学特征。