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首页> 外文期刊>Neuromuscular disorders: NMD >Muscle weakness in respiratory and peripheral skeletal muscles in a mouse model for nebulin-based nemaline myopathy
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Muscle weakness in respiratory and peripheral skeletal muscles in a mouse model for nebulin-based nemaline myopathy

机译：基于星云蛋白的线状肌病小鼠模型中呼吸和外周骨骼肌的肌无力

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摘要

Nemaline myopathy is among the most common non-dystrophic congenital myopathies, and is characterized by the presence of nemaline rods in skeletal muscles fibers, general muscle weakness, and hypotonia. Although respiratory failure is the main cause of death in nemaline myopathy, only little is known regarding the contractile strength of the diaphragm, the main muscle of inspiration. To investigate diaphragm contractility, in the present study we took advantage of a mouse model for nebulin-based nemaline myopathy that we recently developed. In this mouse model, exon 55 of Neb is deleted (Neb(Delta Exon55)), a mutation frequently found in patients. Diaphragm contractility was determined in permeabilized muscle fibers and was compared to the contractility of permeabilized fibers from three peripheral skeletal muscles: soleus, extensor digitorum longus, and gastrocnemius. The force generating capacity of diaphragm muscle fibers of Neb(Delta Exon55) mice was reduced to 25 of wildtype levels, indicating severe contractile weakness. The contractile weakness of diaphragm fibers was more pronounced than that observed in soleus muscle, but not more pronounced than that observed in extensor digitorum longus and gastrocnemius muscles. The reduced muscle contractility was at least partly caused by changes in cross-bridge cycling kinetics which reduced the number of bound cross-bridges. The severe diaphragm weakness likely contributes to the development of respiratory failure in Neb(Delta Exon55) mice and might explain their early, postnatal death.
机译：线状肌病是最常见的非营养不良性先天性肌病之一，其特征是骨骼肌纤维中存在线状杆、全身肌无力和肌张力减退。尽管呼吸衰竭是线状肌病死亡的主要原因，但对膈肌（吸气的主要肌肉）的收缩强度知之甚少。为了研究膈肌收缩力，在本研究中，我们利用了我们最近开发的基于星云蛋白的线状肌病小鼠模型。在这种小鼠模型中，Neb 的外显子 55 被删除（Neb（Delta 外显子 55）），这是一种在患者中经常发现的突变。测定透性肌纤维的膈肌收缩力，并与来自三个外周骨骼肌（比目鱼肌、趾长伸肌和腓肠肌）的透化纤维的收缩力进行比较。Neb（Delta Exon55）小鼠膈肌纤维的发力能力降低至野生型水平的25%，表明收缩力严重无力。膈肌纤维的收缩无力比在比目鱼肌中观察到的更明显，但不比在趾长伸肌和腓肠肌中观察到的更明显。肌肉收缩力的降低至少部分是由于跨桥循环动力学的变化减少了束缚的跨桥的数量。严重的膈肌无力可能导致Neb（Delta Exon55）小鼠呼吸衰竭的发展，并可能解释它们出生后的早期死亡。

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