中国科技核心期刊
CN:31-1600/Q
ISSN:1004-0374
“健康与疾病的免疫”国际学术研讨会通知      关于有网站冒充本刊网站的声明
《生命科学》 2005, (5): 439-
IGF-2r的结构和功能及其在胚胎发育中的作用
龙健儿 *,贺力强,蔡 霞
上海交通大学附属儿童医院医学遗传研究所, 上海200040
摘 要:胰岛素样生长因子-2受体(insulin-like growth factor-2 receptor, IGF-2r)和非阳离子依赖型甘露糖-6-磷酸受体(cation-independent mannose-6-phosphate receptor, CI-MPR)为同一分子,属多结构域跨膜糖蛋白。IGF-2r与阳离子依赖型M6P受体(cation-dependent mannose-6-phosphotase receptor, CD-MPR)共同介导溶酶体酶的分选和转运过程,对溶酶体的形成起重要作用。IGF-2/M6P受体除能与溶酶体酶、TGF-b前体等M6P配体结合外,还能与非糖基化的IGF-2、视黄酸等作用,调节蛋白质的转运和跨膜信号传导等活动。IGF-2r为父源性印迹(paternal imprinted)基因,基因的印迹、表达受基因印迹控制区(imprinting control region, ICR)的甲基化差异修饰所调控。IGF-2r基因表达缺失出现胎儿肥大、心脏和胎盘发育不全、围产期死亡等异常现象,证明IGF-2r在胚胎发育过程中起重要作用。
关键词:IGF-2r;M6P受体;IGF;溶酶体酶;基因印迹;胚胎发育
 
IGF-2r structure, function and its role in the embryonic development
LONG Jian-Er*, HE Li-Qiang, CAI Xia
Shanghai Institute of Medical Genetics, Shanghai Children{$39}s Hospital, Shanghai Jiaotong University, Shanghai 200040, China
Abstract: The insulin-like growth factor-II receptor is a multifunctional single transmembrane glycoprotein with multi-domain, which is identical to the cation-independent mannose-6-phosphate (M6P) receptor. The IGF-II/M6P receptor, along with the cation-dependent M6P (CD-M6P) receptor, mediates the intercellular sorting and trafficking of M6P-containing lysosomal enzymes from the trans-Golgi network (TGN) to lysosomes. In addition to lysosomal enzymes, the IGF-II/M6P receptor also binds a diverse spectrum of other M6P containing proteins, including transforming growth factor-b (TGF-b) precursor, as well as non-M6P-containing retinoic acid and IGF-2, thereby regulates a myriad of biological processes, such as protein trafficking and transmembrane signal transduction, to affect the developmental growth and metablism. IGF-2r gene is generally imprinted on the paternally inherited allele and expressed from maternal allele depending on the imprinting control region (ICR) differentially methylated. The IGF-II/M6P receptor plays a critical role in the embryonic development and is supported by the phenotypes that the disruption of the gene resulted in disproportional overgrowth, particularly of the heart and placenta during post-implantation, and perinatal lethality.
Key words: IGF-2 receptor; M6P receptor; IGF; lysosomal enzymes; gene imprinting; embryonic development
 
首页 | 刊物简介 | 编委会 | 投稿须知 | 广告业务 | 过刊浏览 | 联系我们
中国科学院上海生命科学信息中心《生命科学》编辑部
Copyright © 2012-2015 《生命科学》编辑部 All Rights Reserved.
沪ICP备05033115号-30
您是第2955143 位访问者,欢迎!