Details

Autor(en) / Beteiligte

• Samuelsson, Andrew R.
• Belvindrah, Richard
• Wu, Chuanyue
• Müller, Uli
• Halfter, Willi

Titel

β1-Integrin Signaling is Essential for Lens Fiber Survival

Ist Teil von

• Gene regulation and systems biology, 2007-01, Vol.1, p.117762500700100

Ort / Verlag

London, England: SAGE Publications

Erscheinungsjahr

2007

Quelle

EZB Electronic Journals Library

Beschreibungen/Notizen

• Integrins have been proposed to play a major role in lens morphogenesis. To determine the role of β1-integrin and its down-stream signaling partner, integrin linked kinase (ILK), in lens morphogenesis, eyes of WT mice and mice with a nestin-linked conditional knockout of β1-integrin or ILK were analyzed for defects in lens development. Mice, lacking the genes encoding the p1-integrin subunit (Itgb1) or ILK (Ilk), showed a perinatal degeneration of the lens. Early signs of lens degeneration included vacuolization, random distribution of lens cell nuclei, disrupted fiber morphology and attenuation and separation of the lens capsule. The phenotype became progressively more severe during the first postnatal week eventually leading to the complete loss of the lens. A more severe phenotype was observed in ILK mutants at similar stages. Eyes from embryonic day 13 β1-integrin-mutant embryos showed no obvious signs of lens degeneration, indicating that mutant lens develops normally until peri-recombination. Our findings suggest that β1-integrins and ILK cooperate to control lens cell survival and link lens fibers to the surrounding extracellular matrix. The assembly and integrity of the lens capsule also appears to be reliant on integrin signaling within lens fibers. Extrapolation of these results indicates a novel role of integrins in lens cell-cell adhesions as well as a potential role in the pathogenesis of congenital cataracts.