Gehrig Stefan M, Ryall James G, Schertzer Jonathan D, Lynch Gordon S
Basic and Clinical Myology Laboratory, Department of Physiology, The University of Melbourne, Victoria 3010, Australia.
Exp Physiol. 2008 Nov;93(11):1190-8. doi: 10.1113/expphysiol.2008.042838. Epub 2008 Jun 20.
Contraction-mediated injury is a major contributing factor to the pathophysiology of muscular dystrophy and therefore therapies that can attenuate this type of injury have clinical relevance. Systemic administration of insulin-like growth factor-I (IGF-I) has been shown to improve muscle function in dystrophic mdx mice, an effect associated with a shift towards a more oxidative muscle phenotype and a reduced susceptibility to contraction-mediated damage. The actions of IGF-I in vivo are modulated by IGF binding proteins (IGFBPs), which generally act to inhibit IGF-I signalling. We tested the hypothesis that an analogue of IGF-I (LR IGF-I), which has significantly reduced binding affinity for IGFBPs, would improve the dystrophic pathology by reducing the susceptibility to muscle injury. Dystrophic mdx and wild-type (C57BL/10) mice were administered LR IGF-I continuously ( approximately 1.5 mg kg(-1) day(-1)) via osmotic mini-pump for 4 weeks. Administration of LR IGF-I reduced the susceptibility of extensor digitorum longus, soleus and diaphragm muscles to contraction damage, as evident from lower force deficits after a protocol of lengthening contractions. In contrast to the mechanism of protection conferred by administration of IGF-I, the protection conferred by LR IGF-I was independent of changes in muscle fatigue and oxidative metabolism. This study further indicates that modulation of IGF-I signalling has therapeutic potential for muscular diseases.
收缩介导的损伤是肌营养不良病理生理学的一个主要促成因素,因此能够减轻这类损伤的疗法具有临床意义。已表明,全身性给予胰岛素样生长因子-I(IGF-I)可改善营养不良的mdx小鼠的肌肉功能,这一效应与向更具氧化型的肌肉表型转变以及对收缩介导损伤的易感性降低有关。IGF-I在体内的作用受IGF结合蛋白(IGFBPs)调节,这些蛋白通常起到抑制IGF-I信号传导的作用。我们检验了这样一个假说,即一种对IGFBPs的结合亲和力显著降低的IGF-I类似物(LR IGF-I),会通过降低对肌肉损伤的易感性来改善营养不良病理。通过渗透微型泵给营养不良的mdx小鼠和野生型(C57BL/10)小鼠连续4周给予LR IGF-I(约1.5 mg·kg⁻¹·天⁻¹)。LR IGF-I的给药降低了趾长伸肌、比目鱼肌和膈肌对收缩损伤的易感性,这从延长收缩方案后的较低力量缺损可以明显看出。与给予IGF-I所赋予的保护机制不同,LR IGF-I所赋予的保护与肌肉疲劳和氧化代谢的变化无关。这项研究进一步表明,调节IGF-I信号传导对肌肉疾病具有治疗潜力。