| 多囊蛋白-1的研究进展 |
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| 引用本文： | 李群,李海浪,邓红.多囊蛋白-1的研究进展[J].国际病理科学与临床杂志,2006,26(2):15-149. |
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| 作者姓名： | 李群 李海浪 邓红 |
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| 作者单位： | 东南大学临床医学院儿科,南京,210009;浙江大学医学院病理学与病理生理学系,杭州,310031 |
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| 摘 要： | 多囊蛋白-1（polycystin-1,PC-1）是由多囊肾病基因-1（PKD1）编码的跨膜蛋白，属多囊蛋白家族成员，主要在上皮细胞、内分泌细胞、心肌细胞和骨骼肌细胞上表达。PC-1可与多囊蛋白-2（polycystin-2,PC-2）、踝蛋白、纽蛋白等蛋白结合，参与细胞-细胞、细胞-基质黏附和细胞间信号转导。PC-1可调节肾小管上皮细胞的增殖和分化、介导细胞间的黏附，在肾脏的正常发育和常染色体显性遗传性多囊肾病（ADPKD）发病起重要作用。
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| 关 键 词： | 多囊蛋白-1 结构 功能 常染色体显性遗传性多囊肾病 |
| 文章编号： | 1673-2588(2006)01-0146-04 |
| 收稿时间： | 2005-11-9 |
| 修稿时间： | 2005年11月9日 |
The advances of polycystin-1 |
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| LI Qun,LI Hai-lang,DENG Hong.The advances of polycystin-1[J].Journal of International Pathology and Clinical Medicine,2006,26(2):15-149. |
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| Authors: | LI Qun LI Hai-lang DENG Hong |
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| Institution: | 1. Department of Pediatrics, School of Clinical Medicine, Southeast University, Nanjing 210009, China;2. Department of Pathology and Pathophysiology, Zhejiang University School of Medicine, Hangzhou 310031, China |
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| Abstract: | Polycystin-1(PC-1),the protein product of PKD1 gene,is a transmembrane protein as a member of polycystin family.Its expression is mainly seen in epithelia,endocrine cells,myocardial cells and myocytes of skeletal muscles.PC-1 forms multiprotein complexes with polycystin-2(PC-2),(talin,) vinculin and other proteins,mediating cell-cell/cell-matrix adhesion and signal transduction.It is proposed that PC-1 may regulate the proliferation and differentiation of renal tubule epithelia and cell-cell interactions. It plays an essential role in the development of normal kidney and cyst formation of autosomal dominant polycystic kidney disease(ADPKD). |
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| Keywords: | polycystin-1 structure function autosomal dominant polycystic kidney disease |
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