Role of Mutant TBP in Regulation of Myogenesis on Muscle Satellite Cells
Role of Mutant TBP in Regulation of Myogenesis on Muscle Satellite Cells
复制标题
突变体 TBP 在调节肌卫星细胞的肌生成中的作用
DOI：
10.1007/s11596-019-2099-y
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发表时间：
2019-10
期刊：
影响因子：
--
通讯作者：
Huang SS
中科院分区：
文献类型：
--
作者：
Zhao DM;Zhu SQ;Wang FR;Huang SS
在多聚谷氨酰胺（PolyQ）疾病中，突变蛋白不仅会导致神经系统问题，还会导致外周组织异常。在所有的全身性损害中，骨骼肌营养不良是最严重的。在脊髓性小脑性共济失调17（SCA17）基因敲入（KI）小鼠模型的研究中发现，突变的TATA盒结合蛋白（TBP）降低了与肌源性分化抗原的相互作用，从而降低了骨骼肌结构蛋白的表达，导致肌肉变性。本文研究了突变型TBP在肌细胞发生中的作用。从野生型（WT）和SCA17KI小鼠的胫骨前肌分离单个肌纤维。1TBP18染色证实突变型TBP在SCA17 KI小鼠的肌肉卫星细胞中表达。在BaCl2诱导的TA肌肉损伤中，H&E横截面染色显示，BaCl2处理前后肌原纤维大小无明显变化，WT和SCA17 KI小鼠的集中核无明显差异，表明突变TBP对肌肉再生无明显影响。在体外培养的WT和SCA17KI小鼠原代成肌细胞中，代表性的BrdU免疫染色显示肌卫星细胞的增殖没有显著差异。然后诱导原代成肌细胞分化并对eMyHC进行免疫染色，染色显示WT和SCA1KI小鼠之间的原代成肌细胞分化没有显著差异。我们的研究结果证实，突变TBP对肌发生没有显着影响。
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 BaCl2treatment, 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 micein 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.
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DOI：
10.1038/nrn.2017.92
发表时间：
2017-10
期刊：
Nature reviews. Neuroscience
影响因子：
--
作者：
Paulson HL;Shakkottai VG;Clark HB;Orr HT
通讯作者：
Orr HT
前列腺素F2（α）通过NFATC2依赖性途径刺激骨骼肌细胞的生长。
DOI：
10.1083/jcb.200208085
发表时间：
2003-04-14
期刊：
The Journal of cell biology
影响因子：
--
作者：
Horsley V;Pavlath GK
通讯作者：
Pavlath GK
DOI：
10.1002/0470018860.s00410
发表时间：
2010-01
期刊：
Principles and Practice of Movement Disorders
影响因子：
--
作者：
Joseph Jankovic;Mark Hallett;Michael S. Okun;Cynthia L. Comella;Stanley Fahn;Jennifer Goldman
通讯作者：
Joseph Jankovic;Mark Hallett;Michael S. Okun;Cynthia L. Comella;Stanley Fahn;Jennifer Goldman
影响因子：
2.3
作者：
den Dunnen, W. F. A.
通讯作者：
den Dunnen, W. F. A.
影响因子：
3.5
作者：
Apponi, Luciano H.;Leung, Sara W.;Pavlath, Grace K.
通讯作者：
Pavlath, Grace K.