[HTML][HTML] Disruption of α3 connexin gene leads to proteolysis and cataractogenesis in mice
X Gong, E Li, G Klier, Q Huang, Y Wu, H Lei, NM Kumar… - Cell, 1997 - cell.com
X Gong, E Li, G Klier, Q Huang, Y Wu, H Lei, NM Kumar, J Horwitz, NB Gilula
Cell, 1997•cell.comGap junction channels formed by α 3 (Cx46) and α 8 (Cx50) connexin provide pathways for
communication between the fiber cells in the normal transparent lens. To determine the
specific role of α 3 connexin in vivo, the α 3 connexin gene was disrupted in mice. Although
the absence of α 3 connexin had no obvious influence on the early stages of lens formation
and the differentiation of lens fibers, mice homozygous for the disrupted α 3 gene developed
nuclear cataracts that were associated with the proteolysis of crystallins. This study …
communication between the fiber cells in the normal transparent lens. To determine the
specific role of α 3 connexin in vivo, the α 3 connexin gene was disrupted in mice. Although
the absence of α 3 connexin had no obvious influence on the early stages of lens formation
and the differentiation of lens fibers, mice homozygous for the disrupted α 3 gene developed
nuclear cataracts that were associated with the proteolysis of crystallins. This study …
Abstract
Gap junction channels formed by α3 (Cx46) and α8 (Cx50) connexin provide pathways for communication between the fiber cells in the normal transparent lens. To determine the specific role of α3 connexin in vivo, the α3 connexin gene was disrupted in mice. Although the absence of α3 connexin had no obvious influence on the early stages of lens formation and the differentiation of lens fibers, mice homozygous for the disrupted α3 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.
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