Severely Atrophic Muscle Fibers with Nuclear Clumps Survive many Years in Permanently Denervated Human Muscle

H. Kern, U. Carraro, D. Biral, N. Adami, S. Zampieri
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引用次数: 17

Abstract

After complete lumbar-ischiatic spinal cord injury (SCI) the lower motor neuron (LMN) denervated human muscle fibers lose completely the myofibrillar apparatus and the coil distribution of myonuclei that are relocated in groups (nuclear clumps) in the center of these "severely atrophic" muscle fibers. In our cohort of patients, the "severely atrophic" myofibers are frequent in muscle biopsies harvested three to six years after SCI. Up to two years of LMN denervation the muscle fibers with nuclear clumps are 2 ± 5% (mean ± SD) of the total muscle fibers. The percentage increases to 27 ± 9% between three and six years of denervation (p< 0.001), and then abruptly decrease from the 6th year of LMN denervation onward, when fibrosis takes over to neurogenic muscle atrophy. Immunohistochemical analyses show that nuclear grouping occurs in both fast and slow muscle fibers. These results show that human muscle fibers survive permanent denervation much longer than generally accepted.
具有核团块的严重萎缩肌纤维在永久性失神经的人类肌肉中存活多年
完全性腰坐骨脊髓损伤(SCI)后,下运动神经元(LMN)失神经支配的人肌纤维完全丧失了肌原纤维装置和肌核的线圈分布,肌核在这些“严重萎缩”的肌纤维中心成组地重新定位(核团块)。在我们的患者队列中,在脊髓损伤后3至6年的肌肉活检中,“严重萎缩”的肌纤维是常见的。长达两年的LMN去神经支配,有核团块的肌纤维占肌纤维总数的2±5%(平均±SD)。在去神经支配的3 - 6年间,这一比例增加到27±9% (p< 0.001),然后从LMN去神经支配的第6年开始,当纤维化转变为神经源性肌肉萎缩时,这一比例突然下降。免疫组化分析显示,在快、慢肌纤维中均存在核分组。这些结果表明,人类肌肉纤维在永久性去神经支配中存活的时间比一般认为的要长得多。
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