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First published online May 1, 2006

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Shedding light on lens transparency

Primary and secondary lens fibres are formed by lens fibre cells, and gap junctions between these cells are vital for maintaining lens transparency. Xiaohua Gong and colleagues (p. 2033) have investigated how mutations in the genes that encode gap-junction subunits (which allow the exchange of molecules between cells) predispose to cataract formation. The gap junctions between lens fibre cells consist of two connexin subunits - 3 (Cx46, encoded by Gja3) and 8 (Cx50, encoded by Gja8). The researchers have identified a gain-of-function 8^S50P mutation that causes cataracts in mice. When combined with wild-type 8, 8^S50P inhibits primary lens fibre elongation; when combined with wild-type 3, it disrupts secondary lens fibre differentiation and elongation. The authors hypothesise that the gain-of-function effects of 8^S50P are due to increased channel permeability and fibre-to-fibre transport. The results indicate that gap junctions comprising different connexins regulate lens differentiation in distinct ways, hinting as to why and how different connexin mutations lead to a variety of cataracts.

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Related articles in Development:

Diverse gap junctions modulate distinct mechanisms for fiber cell formation during lens development and cataractogenesis

Chun-hong Xia, Haiquan Liu, Debra Cheung, Catherine Cheng, Eddie Wang, Xin Du, Bruce Beutler, Woo-Kuen Lo, and Xiaohua Gong
Development 2006 133: 2033-2040. [Abstract] [Full Text]  

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Related articles in Development Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Search for Related Content Social Bookmarking                         What's this?