Department of Animal Science, Iowa State University, Ames, Iowa, United States.
Department of Kinesiology, Iowa State University, Ames, Iowa, United States.
Am J Physiol Regul Integr Comp Physiol. 2023 Dec 1;325(6):R692-R711. doi: 10.1152/ajpregu.00246.2022. Epub 2023 Oct 9.
Duchenne muscular dystrophy (DMD), a progressive muscle disease caused by the absence of functional dystrophin protein, is associated with multiple cellular, physiological, and metabolic dysfunctions. As an added complication to the primary insult, obesity/insulin resistance (O/IR) is frequently reported in patients with DMD; however, how IR impacts disease severity is unknown. We hypothesized a high-fat, high-sucrose diet (HFHSD) would induce O/IR, exacerbate disease severity, and cause metabolic alterations in dystrophic mice. To test this hypothesis, we treated 7-wk-old mdx (disease model) and C57 mice with a control diet (CD) or an HFHSD for 15 wk. The HFHSD induced insulin resistance, glucose intolerance, and hyperglycemia in C57 and mdx mice. Of note, mdx mice on CD were also insulin resistant. In addition, visceral adipose tissue weights were increased with HFHSD in C57 and mdx mice though differed by genotype. Serum creatine kinase activity and histopathological analyses using Masson's trichrome staining in the diaphragm indicated muscle damage was driven by dystrophin deficiency but was not augmented by diet. In addition, markers of inflammatory signaling, mitochondrial abundance, and autophagy were impacted by disease but not diet. Despite this, in addition to disease signatures in CD-fed mice, metabolomic and lipidomic analyses demonstrated a HFHSD caused some common changes in C57 and mdx mice and some unique signatures of O/IR within the context of dystrophin deficiency. In total, these data revealed that in mdx mice, 15 wk of HFHSD did not overtly exacerbate muscle injury but further impaired the metabolic status of dystrophic muscle.
杜氏肌营养不良症(DMD)是一种进行性肌肉疾病,由功能性肌营养不良蛋白缺失引起,与多种细胞、生理和代谢功能障碍有关。作为对主要损伤的附加并发症,肥胖/胰岛素抵抗(O/IR)在 DMD 患者中经常被报道;然而,IR 如何影响疾病严重程度尚不清楚。我们假设高脂肪、高蔗糖饮食(HFHSD)会引起 O/IR,加重疾病严重程度,并导致营养不良小鼠发生代谢改变。为了验证这一假设,我们用对照饮食(CD)或 HFHSD 治疗 7 周龄 mdx(疾病模型)和 C57 小鼠 15 周。HFHSD 诱导 C57 和 mdx 小鼠胰岛素抵抗、葡萄糖耐量降低和高血糖。值得注意的是,CD 喂养的 mdx 小鼠也存在胰岛素抵抗。此外,HFHSD 使 C57 和 mdx 小鼠的内脏脂肪组织重量增加,但基因型不同。血清肌酸激酶活性和 Masson 三色染色的组织病理学分析表明,肌肉损伤是由肌营养不良蛋白缺乏引起的,但饮食没有加重。此外,炎症信号、线粒体丰度和自噬的标志物受疾病影响,但不受饮食影响。尽管如此,除了 CD 喂养的小鼠中的疾病特征外,代谢组学和脂质组学分析表明,HFHSD 导致 C57 和 mdx 小鼠发生一些共同变化,并在肌营养不良蛋白缺乏的背景下导致 O/IR 的一些独特特征。总之,这些数据表明,在 mdx 小鼠中,15 周的 HFHSD 并没有明显加重肌肉损伤,但进一步损害了营养不良肌肉的代谢状态。