Role of Mutant TBP in Regulation of Myogenesis on Muscle Satellite Cells  

作　　者：Dong-ming ZHAO Sui-qiang ZHU Fu-rong WANG Shan-shan HUANG 

机构地区：[1]Department of Orthopedics,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430030,China [2]Department of Neurology,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430030,China

出　　处：《Current Medical Science》2019年第5期734-740,共7页当代医学科学（英文）

基　　金：This project was supported by grants from the fundamental Research Funds for the Central Universities(No.2019kfyXKJC075);National Key R&D Program of China(No.2017YFC1310000);National Natural Science Foundation of China(No.81671064,and No.81371222).

摘　　要：In polyglutamine(PolyQ)diseases,mutant proteins cause not only neurological problems but also peripheral tissue abnormalities.Among all systemic damages,skeletal muscle dystrophy is the severest.Previously by studying knock-in(KI)mouse models of spinal cerebellar ataxia 17(SCA17),it was found that mutant TATA box binding protein(TBP)decreases its interaction with myogenic differentiation antigen,thus reducing the expression of skeletal muscle structural proteins and resulting in muscle degeneration.In this paper,the role of mutant TBP in myogenesis was investigated.Single myofibers were isolated from tibialis anterior muscles of wild type(WT)and SCA17KI mice.The 1TBP18 staining confirmed the expression of mutant TBP in muscle satellite cells in SCA17Ki mice.In the BaCl2-induced TA muscle injury,H&E cross-section staining showed no significant change in myofibril size before and after BaCl2 treatment,and there was no significant difference in centralized nuclei between WT and SCA17KI mice,suggesting that mutant TBP had no significant effect on muscle regeneration.In the cultured primary myoblasts from WT and SCA17KI mice in vitro,representative BrdU immunostaining showed no significant difference in proliferation of muscle satellite cells.The primary myoblasts were then induced to differentiate and immunostained for eMyHC,and the staining showed there was no significant difference in differentiation of primary myoblasts between WT and SCA1KI mice.Our findings confirmed that mutant TBP had no significant effect on myogenesis.

关 键 词：TATA box binding protein SPINOCEREBELLAR ATAXIA 17 MYOBLAST MYOGENESIS 

分 类 号：R73[医药卫生—肿瘤]