中国竞彩网

ZHONGKEYUANDONGWUYANJIUSUOHEZUOKAIFAJIEJINGYANGSHIWANGMOBIANXINGBINGDEJIYINZHILIAO

中国竞彩网  JIEJINGYANGSHIWANGMOBIANXING(Bietti crystalline dystrophy, BCD)SHIYILEIHUIDUISHILIJIANKANGZAOCHENGYANZHONGWEIHAIDEDANJIYINYICHUANBING,YIHUANZHEYANDIDEDALIANGHUANGBAISESHANLIANGJIEJINGCHENJIWEIDAIBIAOXINGTEZHENG。TADELINGYIGEXIANZHUTEZHENGSHI,XIANGDUIYUQITARENQUN,BCDZAIDONGYARENQUNZHONGJUYOUXIANZHUZENGGAODEFABINGLV。JUGUJI,ZHONGGUORENQUNZHONGBCDDEFABINGLVYUEWEI1/24000,ZHANQUANBUSHIWANGMOSESUBIANXINGHUANZHEDE1/6ZHI1/10。DADUOSHUBCDHUANZHEYU20-40SUIFABING,CHUXIANYEMANGHESHILIXIAJIANGDENGZHENGZHUANG,ZAI50-60SUIFAZHANWEIFADINGMANGREN。

  SUIRANSHIYILEIWEIHAIYANZHONGDEZHIMANGXINGYICHUANYANBING,DANYUYIXIEXIFANGMEITIGAODUGUANZHUDE“MINGXINGYICHUANBING”XIANGBI,ZHENDUIBCDDEJICHUHELINCHUANGYANJIUDOUJIAOWEIKUIFA。QIJINBCDRENGRANQUEFAYOUXIAODEDONGWUMOXING,DAOZHIYIFANGMIANWUFAJINYIBULEJIEBCDBINGCHENGDEYINGXIANGYINSU;LINGYIFANGMIANYEWUFAYANZHENGQIANZAIDEZHILIAOYAOWU。ZAIYIDALIYISHENGG.B. BiettiSHOUCILINCHUANGGUANCHADAOBINGWEIZHEIZHONGJIBINGMINGMINGDEBASHINIANZHIHOU,BCDYIRANWUYAOKEZHI。

  CYP4V2是人体内的ω-氢基化酶基因,在多种代谢通路中发挥作用。遗传自父方和母方的CYP4V2基因同时发生突变,导致了BCD的发病。相对于正常水平而言,BCD患者体内的脂类水平有着显著异常。通过敲除小鼠中包含整个Cyp4v3CYP4V2的小鼠同源基因)的完整基因座,中科院动物所研究团队成功构建了Cyp4v3完全缺失的小鼠模型。进一步研究发现,高脂肪饲料喂养的Cyp4v3中国竞彩网缺失小鼠模型,展现出眼底结晶和视力功能下降的双重BCD疾病表型。作为六大营养物质之一,脂肪在日常饮食中无法避免。以上的研究结果表明,过高的脂类水平不仅是BCD疾病的异常表征,还是能加速BCD疾病进程的危险因素。这提示了控制日常脂肪摄入,可能是改善BCD疾病护理、延缓疾病进程的重要手段。

  基因治疗(Gene Therapy)是将外源基因导入细胞,纠正或补偿因基因缺陷或基因表达异常引起的疾病的新型治疗手段,在对遗传性疾病的治疗上具有重大潜力。动物所研究团队通过与北京协和医院合作,将腺相关病毒 (Adenovirus associated virus, AAV)包装的人CYP4V2基因注射到高脂肪饲喂的Cyp4v3敲除小鼠模型眼底,进一步验证基因治疗对BCD的适用性。研究发现,经过基因治疗的小鼠眼底厚度显著增加,视觉功能(眼底的电生理反应水平)也得到改善。这些结果首次在活体动物模型中展现了基因治疗对于BCD治疗的有效性,为BCD患者带来了希望的曙光。

  XIANGGUANCHENGGUOYU2020NIAN 6YUE1RIZAIGUOJIXUESHUQIKANGene TherapyFABIAO[1]。GAIYANJIUGONGZUOYOUZHONGGUOKEXUEYUANDONGWUYANJIUSUO、ZHONGGUOKEXUEYUANGANXIBAOYUZAISHENGYIXUECHUANGXINYANJIUYUANHEBEIJINGXIEHEYIYUANHEZUOWANCHENG。ZHONGGUOKEXUEYUANDONGWUYANJIUSUOLIWEIYANJIUYUANHEBEIJINGXIEHEYIYUANSUIRUIFANGJIAOSHOUWEILUNWENDETONGXUNZUOZHE;QUBIN、WUSHIJING、JIAOGUANYI、ZOUXUAN、LIZHIKUN、GUOLUWEIGONGTONGDIYIZUOZHE。GAIYANJIUSHOUDAOKEJIBU、JIJINWEIJIZHONGKEYUANZHANLVEKEJIXIANDAOZHUANXIANGDENGXIANGMUDEZIZHU。

TU1 GAOZHIYINSHIJIASUBCDJIBINGJINCHENG,JIYINZHILIAOGAISHANMOXINGSHUSHIJUEGONGNENG

  LUNWENLIANJIE:

  1. Qu B, Wu S, Jiao G, Zou X, Li Z, Guo L et al.中国竞彩网 Treating bietti crystalline dystrophy in a high-fat diet-exacerbated murine model using gene therapy. Gene Therapy. 2020. https://doi.org/10.1038/s41434-020-0159-3

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