Disruption of alpha(3) connexin gene leads to proteolysis and cataractogenesis in mice
Disruption of alpha(3) connexin gene leads to proteolysis and cataractogenesis in mice
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DOI：
10.1016/s0092-8674(00)80471-7
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发表时间：
1997-12-12
期刊：
影响因子：
64.5
通讯作者：
Gilula, NB
中科院分区：
文献类型：
--
作者：
Gong, XH;Li, E;Gilula, NB
由α(3)(Cx46)和α(8)(CX50)连接蛋白形成的缝隙连接通道为正常透明晶状体中纤维细胞之间的通讯提供了途径。为了确定α(3)连接蛋白在体内的特定作用，我们在小鼠体内阻断了α(3)连接蛋白基因。虽然α(3)连接蛋白的缺失对晶状体形成的早期阶段和晶状体纤维的分化没有明显的影响，但突变的α(3)基因纯合的小鼠患上了与晶状体蛋白分解有关的核性白内障。本研究通过为晶状体膜和细胞质蛋白的正确组织提供细胞-细胞信号通路或结构成分，确立了缝隙连接在维持正常晶状体透明度方面的重要性。
Gap junction channels formed by alpha(3) (Cx46) and alpha(8) (Cx50) connexin provide pathways for communication between the fiber cells in the normal transparent lens. To determine the specific role of alpha(3) connexin in vivo, the alpha(3) connexin gene was disrupted in mice. Although the absence of alpha(3) connexin had no obvious influence on the early stages of lens formation and the differentiation of lens fibers, mice homozygous for the disrupted alpha(3) gene developed nuclear cataracts that were associated with the proteolysis of crystallins. This study establishes the importance of gap junctions in maintaining normal lens transparency by providing a cell-cell signaling pathway or structural component for the proper organization of lens membrane and cytoplasmic proteins.