Nerve-dependent changes in skeletal muscle myosin heavy chain after experimental denervation and cross-reinnervation and in a demyelinating mouse model of Charcot-Marie-Tooth disease type 1A.
Nerve-dependent changes in skeletal muscle myosin heavy chain after experimental denervation and cross-reinnervation and in a demyelinating mouse model of Charcot-Marie-Tooth disease type 1A.
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DOI：
10.1002/mus.21106
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发表时间：
2008-12
期刊：
影响因子：
3.4
通讯作者：
Hughes, Simon M.
中科院分区：
文献类型：
--
作者：
Maggs, Alison M.;Huxley, Clare;Hughes, Simon M.
关键词：
神经支配调节脊椎动物肌纤维的收缩特性，部分是通过电活动对不同肌球蛋白表达的影响。在这里，我们分析了神经支配的作用，在调节的一般，成熟和成年形式的啮齿动物慢肌球蛋白重链（MyHC）的存在下定义的不同的抗原表位的积累。去神经支配增加了表达一般慢型MyHC的纤维数量，但减少了成人慢型MyHC表位。快神经对慢肌的交叉再支配导致表达快肌复合物的纤维数量增加。在这两种情况下，有一个增加的纤维，表达慢和快IIA MyHC，但没有成人慢MyHC表位。这些数据表明，神经支配是需要的成熟和维持多样性的慢和快的纤维。慢MyHC表位转换序列是一种有用的生物标志物，它可能在肌纤维功能的神经依赖性变化中发挥重要作用。我们将这种详细的肌肉分析应用于人类运动和感觉神经病IA的转基因小鼠模型，也称为Charcot-Marie-Tooth疾病1A型（CMT 1A），其中一些运动神经元的电传导由于脱髓鞘而很差。小鼠显示出一些肌纤维的萎缩以及慢和快MyHC表位表达的变化，这表明即使在早期阶段，快速运动神经元对肌纤维的神经支配也在逐渐增加。这些疾病的发病机制的早期变化的潜在作用进行了讨论。
Innervation regulates the contractile properties of vertebrate muscle fibers, in part through the effect of electrical activity on expression of distinct myosins. Here we analyse the role of innervation in regulating the accumulation of the general, maturational and adult forms of rodent slow myosin heavy chain (MyHC) that are defined by the presence of distinct antigenic epitopes. Denervation increases the number of fibers that express general slow MyHC, but it decreases the adult slow MyHC epitope. Cross-reinnervation of slow muscle by a fast nerve leads to an increase in the number of fibers that express fast MyHC. In both cases, there is an increase in fibers that express slow and fast IIA MyHCs but without the adult slow MyHC epitope. The data suggest that innervation is required for maturation and maintenance of diversity of both slow and fast fibers. The sequence of slow MyHC epitope transitions is a useful biomarker, and it may play a significant role during nerve-dependent changes in muscle fiber function. We applied this detailed muscle analysis to a transgenic mouse model of Human Motor and Sensory Neuropathy IA, also known as Charcot-Marie-Tooth disease Type 1A (CMT1A), in which electrical conduction in some motor neurons is poor due to demyelination. The mice display atrophy of some muscle fibers and changes in slow and fast MyHC epitope expression suggestive of a progressive increase in innervation of muscle fibers by fast motor neurons, even at early stages. The potential role of these early changes in disease pathogenesis is discussed.
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在胚胎发育中肌肉纤维形成过程中肌球蛋白重链缓慢链表达缓慢的肌球蛋白重链表达的证据。
影响因子：
7.8
作者：
Cho, M;Webster, S G;Blau, H M
通讯作者：
Blau, H M
影响因子：
--
作者：
BUTLER, J;COSMOS, E;BRIERLEY, J
通讯作者：
BRIERLEY, J
DOI：
10.1083/jcb.103.3.995
发表时间：
1986-09
期刊：
The Journal of cell biology
影响因子：
--
作者：
Ecob-Prince MS;Jenkison M;Butler-Browne GS;Whalen RG
通讯作者：
Whalen RG
影响因子：
5.5
作者：
BULLER, AJ;ECCLES, JC;ECCLES, RM
通讯作者：
ECCLES, RM
DOI：
10.1152/ajpcell.1998.275.4.c1124
发表时间：
1998-10-01
影响因子：
5.5
作者：
Dupont-Versteegden, EE;Houlé, JD;Peterson, CA
通讯作者：
Peterson, CA