Section of Pathology, Department of Biomedical and Biotechnological Sciences, School of Medicine, University of Catania, 95123 Catania, Italy.
Italian Center for the Study of Osteopathy (CSDOI), 95124 Catania, Italy.
Int J Mol Sci. 2024 Oct 21;25(20):11310. doi: 10.3390/ijms252011310.
Muscular dystrophies (MDs) are genetically heterogeneous diseases characterized by primary skeletal muscle atrophy. The collapse of muscle structure and irreversible degeneration of tissues promote the occurrence of comorbidities, including cardiomyopathy and respiratory failure. Mitochondrial dysfunction leads to inflammation, fibrosis, and adipogenic cellular infiltrates that exacerbate the symptomatology of MD patients. Gastrointestinal disorders and metabolic anomalies are common in MD patients and may be determined by the interaction between the intestine and its microbiota. Therefore, the gut-muscle axis is one of the actors involved in the spread of inflammatory signals to all muscles. In this review, we aim to examine in depth how intestinal dysbiosis can modulate the metabolic state, the immune response, and mitochondrial biogenesis in the course and progression of the most investigated MDs such as Duchenne Muscular Dystrophy (DMD) and Myotonic Dystrophy (MD1), to better identify gut microbiota metabolites working as therapeutic adjuvants to improve symptoms of MD.
肌肉萎缩症（MDs）是一组具有遗传异质性的疾病，其特征为主要的骨骼肌萎缩。肌肉结构的崩溃和组织的不可逆转退化促使合并症的发生，包括心肌病和呼吸衰竭。线粒体功能障碍导致炎症、纤维化和脂肪生成细胞浸润，从而加重 MD 患者的症状。胃肠道疾病和代谢异常在 MD 患者中很常见，这可能取决于肠道及其微生物群的相互作用。因此，肠-肌肉轴是将炎症信号传播到所有肌肉的参与者之一。在这篇综述中，我们旨在深入研究肠道菌群失调如何在最受关注的 MD 如杜氏肌营养不良症（DMD）和强直性肌营养不良症（MD1）的发生和进展过程中调节代谢状态、免疫反应和线粒体生物发生，以更好地识别作为治疗佐剂的肠道微生物群代谢物，改善 MD 的症状。
