[1]周乐波),王雪晶),丁雪冰),等.肌萎缩侧索硬化症患者骨骼肌神经末梢中磷酸化TAR DNA结合蛋白43和泛素的表达[J].郑州大学学报(医学版),2019,(02):227-231.[doi:10.13705/j.issn.1671-6825.2018.08.019]
 ZHOU Lebo),WANG Xuejing),DING Xuebing),et al.Detection of phosphorylated TDP-43 and ubiquitin in nerve terminals of skeletal muscle in patients with amyotrophic lateral sclerosis[J].JOURNAL OF ZHENGZHOU UNIVERSITY(MEDICAL SCIENCES),2019,(02):227-231.[doi:10.13705/j.issn.1671-6825.2018.08.019]
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肌萎缩侧索硬化症患者骨骼肌神经末梢中磷酸化TAR DNA结合蛋白43和泛素的表达()
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《郑州大学学报(医学版)》[ISSN:1671-6825/CN:41-1340/R]

卷:
期数:
2019年02期
页码:
227-231
栏目:
应用研究
出版日期:
2019-03-20

文章信息/Info

Title:
Detection of phosphorylated TDP-43 and ubiquitin in nerve terminals of skeletal muscle in patients with amyotrophic lateral sclerosis
作者:
周乐波12)王雪晶12)丁雪冰12)姜晓懿12)滕军放12)
1)郑州大学第一附属医院神经内科 郑州 450052 2)河南省高等学校临床医学重点学科开放实验室 郑州 450052
Author(s):
ZHOU Lebo12)WANG Xuejing12)DING Xuebing12)JIANG Xiaoyi12)TENG Junfang12)
1)Department of Neurology, the First Affiliated Hospital, Zhengzhou University,Zhengzhou 450052 2)Key-Disciplines Laboratory for Clinical Medicine of Henan Province, Zhengzhou 450052
关键词:
肌萎缩侧索硬化症 肌肉活检 TAR DNA结合蛋白43 自噬-溶酶体系统 泛素-蛋白酶体系统
Keywords:
amyotrophic lateral sclerosis muscle biopsy TAR DNA-binding protein 43 autophagy-lysosomal system ubiquitin-proteasome system
分类号:
R744.8
DOI:
10.13705/j.issn.1671-6825.2018.08.019
摘要:
目的:检测肌萎缩侧索硬化症(ALS)患者骨骼肌神经末梢中磷酸化TAR DNA结合蛋白43(pTDP-43)和泛素(Ub)的表达情况,探讨其在ALS发病中的作用机制。方法:收集30例ALS患者及22例对照的骨骼肌标本,采用免疫组化法检测pTDP-43及Ub的表达情况。结果:ALS组、对照组pTDP-43、Ub阳性表达率分别为46.7%、0.0%和60.0%、0.0%,差异有统计学意义(P<0.001)。结论:骨骼肌神经末梢是部分ALS患者除神经元及神经胶质细胞以外的pTDP-43病理沉积部位,可能参与了ALS的发病。
Abstract:
Aim:To investigate the role of phosphorylated TAR DNA-binding protein 43(pTDP-43)and ubiquitin(Ub)in pathogenesis and mechanisms of amyotrophic lateral sclerosis(ALS)by detecting pTDP-43 and Ub in the muscle biopsy.Methods:A total of 52 skeletal muscle samples from 30 ALS patients and 22 normal controls were collected. Immunohistochemistry was used to verify the pathological expressions of pTDP-43 and Ub in muscle tissue.Results:The positive expression rates of pTDP-43 and Ub in ALS group and normal control group were 46.7%, 0.0%, and 60.0%, 0.0%, respectively(P<0.001).Conclusion:Nerve terminals in skeletal muscle are additional sites besides neuron and neurogliocyte of pTDP-43 pathology in some ALS patients, which may play a role in the pathogenesis of ALS.

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相似文献/References:

[1]谢南昌,王亚丽,徐洪亮,等.免疫性坏死性肌病一例*[J].郑州大学学报(医学版),2016,(06):799.

备注/Memo

备注/Memo:
【基金项目】国家自然科学基金资助面上项目(81671267)
【作者简介】滕军放,通信作者,男,1960年11月生,博士,教授,主任医师,研究方向:神经退行性疾病,E-mail:13838210077@163.com
更新日期/Last Update: 2019-03-20