Significance of phosphatidyl inositol 3-kinase expression in amyotrophic lateral sclerosis atrophy muscle fibers
10.3760/cma.j.issn.1671-8925.2014.02.014
- VernacularTitle:磷脂酰肌醇3-激酶在ALS萎缩肌纤维中表达的意义
- Author:
Hongrui SHEN
1
;
Taoran JIN
;
Zhe ZHAO
;
Jing HU
Author Information
1. 河北医科大学第三医院神经肌肉病科
- Keywords:
Amyotrophic lateral sclerosis;
Skeletal muscle biopsy;
Phosphatidylinositol 3-kinase;
Mitochondria;
Apoptosis
- From:
Chinese Journal of Neuromedicine
2014;13(2):173-176
- CountryChina
- Language:Chinese
-
Abstract:
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.
