中华神经医学杂志
中華神經醫學雜誌
중화신경의학잡지
CHINESE JOURNAL OF NEUROMEDICINE
2014年
2期
173-176
,共4页
沈宏锐%靳陶然%赵哲%胡静
瀋宏銳%靳陶然%趙哲%鬍靜
침굉예%근도연%조철%호정
肌萎缩侧索硬化%骨骼肌活检%磷脂酰肌醇3-激酶%线粒体%细胞凋亡
肌萎縮側索硬化%骨骼肌活檢%燐脂酰肌醇3-激酶%線粒體%細胞凋亡
기위축측색경화%골격기활검%린지선기순3-격매%선립체%세포조망
Amyotrophic lateral sclerosis%Skeletal muscle biopsy%Phosphatidylinositol 3-kinase%Mitochondria%Apoptosis
目的 研究磷脂酰肌醇3-激酶(PI3K)在肌萎缩侧索硬化(ALS)患者骨骼肌细胞中的表达及意义.探讨ALS肌纤维萎缩的发病机制. 方法 选取河北医科大学第三医院神经肌肉病科住院部自2005年6月至2013年1月收治的90例ALS患者临床资料,分析临床、血肌酸激酶及电生理特点.全部患者行开放式骨骼肌活检,冰冻连续切片,组织化学、酶学染色及PI3K、神经细胞黏附分子(NCAM)免疫组织化学染色病理分析,电镜观察其超微结构. 结果 (1)ALS患者骨骼肌病理可见大量小角化肌纤维、萎缩肌纤维和核聚集现象.(2)PI3K在萎缩肌纤维膜上呈阳性表达,NCAM在萎缩肌纤维中阴性表达.(3)电镜下萎缩肌纤维中存在典型的线粒体形态异常. 结论 (1)ALS骨骼肌细胞存在线粒体结构和功能异常.(2)ALS的肌纤维萎缩与肌细胞凋亡机制有关.(3)PI3K的异常表达对ALS的诊断或有意义.
目的 研究燐脂酰肌醇3-激酶(PI3K)在肌萎縮側索硬化(ALS)患者骨骼肌細胞中的錶達及意義.探討ALS肌纖維萎縮的髮病機製. 方法 選取河北醫科大學第三醫院神經肌肉病科住院部自2005年6月至2013年1月收治的90例ALS患者臨床資料,分析臨床、血肌痠激酶及電生理特點.全部患者行開放式骨骼肌活檢,冰凍連續切片,組織化學、酶學染色及PI3K、神經細胞黏附分子(NCAM)免疫組織化學染色病理分析,電鏡觀察其超微結構. 結果 (1)ALS患者骨骼肌病理可見大量小角化肌纖維、萎縮肌纖維和覈聚集現象.(2)PI3K在萎縮肌纖維膜上呈暘性錶達,NCAM在萎縮肌纖維中陰性錶達.(3)電鏡下萎縮肌纖維中存在典型的線粒體形態異常. 結論 (1)ALS骨骼肌細胞存在線粒體結構和功能異常.(2)ALS的肌纖維萎縮與肌細胞凋亡機製有關.(3)PI3K的異常錶達對ALS的診斷或有意義.
목적 연구린지선기순3-격매(PI3K)재기위축측색경화(ALS)환자골격기세포중적표체급의의.탐토ALS기섬유위축적발병궤제. 방법 선취하북의과대학제삼의원신경기육병과주원부자2005년6월지2013년1월수치적90례ALS환자림상자료,분석림상、혈기산격매급전생리특점.전부환자행개방식골격기활검,빙동련속절편,조직화학、매학염색급PI3K、신경세포점부분자(NCAM)면역조직화학염색병리분석,전경관찰기초미결구. 결과 (1)ALS환자골격기병리가견대량소각화기섬유、위축기섬유화핵취집현상.(2)PI3K재위축기섬유막상정양성표체,NCAM재위축기섬유중음성표체.(3)전경하위축기섬유중존재전형적선립체형태이상. 결론 (1)ALS골격기세포존재선립체결구화공능이상.(2)ALS적기섬유위축여기세포조망궤제유관.(3)PI3K적이상표체대ALS적진단혹유의의.
Objective To study the significance of phosphatidyl inositol 3-kinase (PI3K) expression in amyotrophic lateral sclerosis (ALS) atrophy muscle fibers and investigate the pathogenesis of muscle fiber atrophy.Methods The clinical data of 90 ALS inpatients,admitted to our hospital from June 2005 to January 2013,were collected; and the features of clinical manifestations,creatine kinase level and electrophysiology were investigated.All patients accepted open muscle biopsy; the frozen tissues were performed histochemical,enzymology,anti-PI3K and nerve cell adhesion molecules (NCAM) monoclonal antibody immunohistochemistry staining; the pathological and ultrastructure characteristics were observed.Results A lot of small angular fibers,atrophy fibers and nuclear clump grouping were observed in skeletal muscle samples of ALS.PI3K positively expressed in sarcolemma of atrophy fibers,while NCAM negatively expressed.The pathologic changes of ultrastructure in transmission electromicroscope illustrated ALS atrophy fibers,having typical mitochondria paramorphia changes.Conclusion Mitochondria paramorphia changes and dysfunction are observed in ALS muscle fibers; atrophy fibers of ALS are related to muscle fiber apoptosis.