Details

Autor(en) / Beteiligte

• Tam, Oliver H.
• Pennisi, David
• Wilkinson, Lorine
• Little, Melissa H.
• Wazin, Fatima
• Wan, Victor L.
• Lovicu, Frank J.

Titel

Crim1 is required for maintenance of the ocular lens epithelium

Ist Teil von

• Experimental eye research, 2018-05, Vol.170, p.58-66

Ort / Verlag

England: Elsevier Ltd

Erscheinungsjahr

2018

Quelle

Access via ScienceDirect (Elsevier)

Beschreibungen/Notizen

• The development and growth of the vertebrate ocular lens is dependent on the regulated proliferation of an anterior monolayer of epithelial cells, and their subsequent differentiation into elongate fiber cells. The growth factor rich ocular media that bathes the lens mediates these cellular processes, and their respective intracellular signaling pathways are in turn regulated to ensure that the proper lens architecture is maintained. Recent studies have proposed that Cysteine Rich Motor Neuron 1 (Crim1), a transmembrane protein involved in organogenesis of many tissues, might influence cell adhesion, polarity and proliferation in the lens by regulating integrin-signaling. Here, we characterise the lens and eyes of the Crim1KST264 mutant mice, and show that the loss of Crim1 function in the ocular tissues results in inappropriate differentiation of the lens epithelium into fiber cells. Furthermore, restoration of Crim1 levels in just the lens tissue of Crim1KST264 mice is sufficient to ameliorate most of the dysgenesis observed in the mutant animals. Based on our findings, we propose that tight regulation of Crim1 activity is required for maintenance of the lens epithelium, and its depletion leads to ectopic differentiation into fiber cells, dramatically altering lens structure and ultimately leading to microphthalmia and aphakia. •Crim1-deficiency in lens leads to inappropriate differentiation of the epithelium into fiber cells resulting in aphakia.•Crim1-deficiency in eye results in vitreal hyaloid vasculature dysplasia, leading to impaired primary fiber cell elongation.•Restoration of Crim1 levels in lens of Crim1 mutants ameliorates the lens epithelial phenotype.•Tight regulation of Crim1 activity is required for maintenance of the lens epithelium.