Specific isomyosin proportions in hyperexcitable and physiologically denervated mouse muscle
Specific isomyosin proportions in hyperexcitable and physiologically denervated mouse muscle
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DOI：
10.1016/s0014-5793(04)00179-6
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发表时间：
2004-03-12
期刊：
影响因子：
3.5
通讯作者：
Jockusch, H
中科院分区：
文献类型：
--
作者：
Agbulut, O;Noirez, P;Jockusch, H
在这里，我们表明，高分辨率十二烷基硫酸钠凝胶电泳，肌球蛋白重链（MyHC）亚型的小鼠肌肉的比例是专门转移遗传性神经肌肉疾病。在野生型和营养不良的MDX胫骨前肌（TA）中，约60%的MyHC是IIB，30% IIX，至多10% IIA和< 2% I型（慢）。在肌强直性快肌中，过度兴奋导致MyHC IIB的急剧减少，其由IIA补偿。慢肌，如比目鱼肌和横膈膜，肌强直只会轻微改变。脊髓性肌萎缩症（SMA）“摇摆”小鼠TA的MyHC模式转变为更快的表型，近90%为IIB。在SMA突变体“肌肉缺陷”中，所有四种成人异肌球蛋白在TA中表达。这些发现可能与未来在小鼠疾病模型和人类患者中诊断神经系统疾病有关。(C)2004年由Elsevier B.V.代表欧洲生物化学学会联合会出版。
We show here, by high resolution sodium dodecyl sulfate gel electrophoresis, that the proportions of myosin heavy chain (MyHC) isoforms of mouse muscles are specifically shifted by hereditary neuromuscular diseases. In wild-type and dystrophic MDX anterior tibial muscle (TA) about 60% of the MyHC is IIB, 30% IIX, at most 10% IIA and < 2% type I (slow). In myotonic fast muscles, hyperexcitability leads to a drastic reduction of MyHC IIB which is compensated by IIA. Slow muscles, like soleus and diaphragm, were only marginally changed by myotonia. The MyHC pattern of TA of spinal muscular atrophy (SMA) 'wobbler' mice is shifted to a faster phenotype, with nearly 90% IIB. In the SMA mutant 'muscle deficient', all four adult isomyosins are expressed in the TA. These findings may be relevant for the future diagnosis of neurological disorders both in mouse disease models and in human patients. (C) 2004 Published by Elsevier B.V. on behalf of the Federation of European Biochemical Societies.