Mitf-M基因突变对猪耳蜗血管纹发育的影响  

Effects of Mitf-M Mutation on Stria Vascularis (SV) in Swine Cochlea

作　　者：陈林军[1] 邱士伟 龙熙[2] 陈磊[2] 唐朝颖 塞娜[1] 郭维维[1] 杨仕明[1] 韩维举[1] CHEN Linjun;QIU Shiwei;LONG Xi;CHEN Lei;TANG Chaoying;SAI Na;GUO Weiwei;YANG Shiming;HAN Wei ju(Department of Otolaryngology Head&Neck Surgery,Chinese PLA General Hospital;Beijing Key Laboratory of Hearing Impairment(Chinese PLA Medical School),Ministry of Education;Beijing Key Laboratory of Hearing Impairment Prevention and Treatment,Beijing 100853,China;Chongqing Academy of Animal Science,Chongqing 402460,China)

机构地区：[1]中国人民解放军总医院耳鼻咽喉头颈外科,聋病教育部重点实验室,聋病防治北京市重点实验室,军事声损伤防护实验室,北京100853 [2]重庆市畜牧科学院,重庆402460

出　　处：《中华耳科学杂志》2018年第4期483-487,共5页Chinese Journal of Otology

基　　金：国家自然科学基金面上项目(No:81770992,81470683,81670940);全军后勤重点项目(BWS14J045);首都卫生发展科研专项重点攻关项目(2016-1-5014);军事医学创新工程和青年培育项目(16QNP133);北京市属医院科研培育计划项目(PX2017046);生物医药与生命科学创新培育研究(Z161100000116038);北京市自然科学基金青年项目,7184202;首都医科大学科研培育基金PYZ2017111.

摘　　要：目的研究Mitf-M基因突变是否会引起血管纹毛细血管、边缘细胞的变化,从而进一步了解Mitf-M基因突变引起Waardenburg综合征的病变机制。方法取出生后7d、10d、30d、40d的野生型和Mitf-M突变型猪共24头,分离解剖耳蜗外侧壁,运用免疫组织化学技术染色,观察野生型和Mitf-M突变型猪耳蜗血管纹毛细血管、边缘细胞在出生后不同时期的免疫荧光的差异。结果 Mitf-M突变型猪与野生型猪在出生后7d、10d及30d,血管纹毛细血管之间没有明显差异,而在40d,Mitf-M突变型猪相对野生型猪的血管纹毛细血管出现明显的减少,并有统计学意义。在7d、40d,Mitf-M突变型猪耳蜗血管纹边缘细胞的细胞骨架均无明显破坏,边缘细胞数量之间没有明显差异。结论 Mitf-M基因突变不引起血管纹边缘细胞骨架的破坏及边缘细胞数量的减少,且Mitf-M突变型猪在出生后早期30d内血管纹毛细血管没有明显变化,在40d后Mitf-M突变型猪较野生型猪的耳蜗血管纹毛细血管出现明显的减少。Objectives We aim to find out whether Mitf-M gene mutation affects stria capillaries and marginal cells in the swine cochlear stria vascularis to better understand the patheogenic mechanism of Waardenburg syndrome.Methods A total of 24 wild type and mutant swines were selected from P7,P10,P30 and P40 after birth.Cochlear samples were obtained and immunohistochemical staining was used to detect the difference in vascular capillaries and marginal cells.Results There were no significant differences in stria capillary at P7 and P10,but at P40 the mutant swines showed a significant decrease of stria capillaries.Moreover,at P7 and P40,the cytoskeleton of stria stria’marginal cells in mutant swines showed no obvious damage,with no significant decrease in the number of marginal cells.Conclusion Mitf-M mutation only causes a decrease of intermediate cells in swine stria vascularis and no damage to the cytoskeleton and number of marginal cells.While there is no significant change in stria capillaris before P30,there is a significant decrease in stria capillaries at P40 in mutant swines.

关 键 词：MITF 血管纹 边缘细胞 血管纹毛细血管 猪 

分 类 号：R764[医药卫生—耳鼻咽喉科]