肌萎缩侧索硬化
内科学
内分泌学
运动神经元
发病机制
神经再支配
生物
神经肌肉疾病
分泌物
进行性肌萎缩
下运动神经元
医学
疾病
神经科学
作者
Frederik J. Steyn,Katie J. Lee,Matthew J. Fogarty,Johannes D. Veldhuis,Pamela McCombe,Mark C. Bellingham,Shyuan T. Ngo,Chen Chen
出处
期刊:Endocrinology
[The Endocrine Society]
日期:2013-10-10
卷期号:154 (12): 4695-4706
被引量:25
摘要
GH deficiency is thought to be involved in the pathogenesis of amyotrophic lateral sclerosis (ALS). However, therapy with GH and/or IGF-I has not shown benefit. To gain a better understanding of the role of GH secretion in ALS pathogenesis, we assessed endogenous GH secretion in wild-type and hSOD1G93A mice throughout the course of ALS disease. Male wild-type and hSOD1G93A mice were studied at the presymptomatic, onset, and end stages of disease. To assess the pathological features of disease, we measured motor neuron number and neuromuscular innervation. We report that GH secretion profile varies at different stages of disease progression in hSOD1G93A mice; compared with age-matched controls, GH secretion is unchanged prior to the onset of disease symptoms, elevated at the onset of disease symptoms, and reduced at the end stage of disease. In hSOD1G93A mice at the onset of disease, GH secretion is positively correlated with the percentage of neuromuscular innervation but not with motor neuron number. Moreover, this occurs in parallel with an elevation in the expression of muscle IGF-I relative to controls. Our data imply that increased GH secretion at symptom onset may be an endogenous endocrine response to increase the local production of muscle IGF-I to stimulate reinnervation of muscle, but that in the latter stages of disease this response no longer occurs.
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