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多囊蛋白,钙离子信号转导与常染色体显性遗传性多囊肾病
引用本文:肖良,张静,赵海丹. 多囊蛋白,钙离子信号转导与常染色体显性遗传性多囊肾病[J]. 医学综述, 2006, 12(5): 259-261
作者姓名:肖良  张静  赵海丹
作者单位:1. 海军总医院肾内科,北京,100037
2. 华北煤炭医学院临床医学2000级9班,河北,唐山,063000
摘    要:多囊肾疾病是由多囊蛋白-1或-2失能导致的,其囊肿的发生涉及到上皮细胞的过度增殖、轻度的分化不良以及囊液的积聚。多囊蛋白-1和多囊蛋白-2构成多囊蛋白离子通道复合物,参与Ca~(2+)信号转导,该通道复合物的病变直接引起细胞内Ca~(2+)调节的失衡导致囊肿的形成。

关 键 词:常染色体显性多囊肾病  多囊蛋白  钙离子信号转导  机械信号转导
文章编号:1006-2084(2006)05-0259-03
收稿时间:2005-05-01
修稿时间:2006-01-06

Polycystins, Calcium Signaling, and Autosomal Dominant Polycystic Kidney Diseases
XIAO Liang,ZHANG Jing,ZHAO Hai-dan. Polycystins, Calcium Signaling, and Autosomal Dominant Polycystic Kidney Diseases[J]. Medical Recapitulate, 2006, 12(5): 259-261
Authors:XIAO Liang  ZHANG Jing  ZHAO Hai-dan
Affiliation:1. Department of Nephrology of the Navy General Hospital, Beijing 100037; 2. North China Coal Medical College, Tangshan 063000
Abstract:The autosomal dominant polycystic kidney disease result from disability of polycystin-1 or polycystin-2.The pathogenesis of cyst formation is thought to relate to over-proliferation of epithelial cells, mild dedifferentiation, and fluid accumulation. These proteins are thought to function together polycystin ion channel complex which may participate in transduction of Ca~(2+) signals.Pathologic changes of the ion channel complex can cause directhy regulation disturbance of intracellular Ca~(2+) and result in cyst formation.
Keywords:Autosomal dominant polycystic kidney disease  Polycystins  Ca~(2+) signaling  Mechanotransduction
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