PMC:5985359 / 23285-24403 JSONTXT

{"project":"2_test","denotations":[{"id":"29526280-27381832-2044781","span":{"begin":417,"end":422},"obj":"27381832"},{"id":"29526280-27196322-2044782","span":{"begin":424,"end":426},"obj":"27196322"},{"id":"29526280-25484056-2044783","span":{"begin":428,"end":430},"obj":"25484056"},{"id":"29526280-24268186-2044784","span":{"begin":518,"end":520},"obj":"24268186"},{"id":"29526280-23641909-2044785","span":{"begin":522,"end":524},"obj":"23641909"},{"id":"29526280-22194824-2044786","span":{"begin":526,"end":528},"obj":"22194824"},{"id":"29526280-25593321-2044787","span":{"begin":836,"end":838},"obj":"25593321"},{"id":"29526280-25722209-2044788","span":{"begin":840,"end":842},"obj":"25722209"}],"text":"On this basis, we tested the potential of using primary CECs, derived from tissue excised during endothelial keratoplasty, to investigate CTG18.1 expansion-associated pathology. The native “endothelial-like” properties of the CECs were confirmed by ICC and a variety of endothelial markers including sodium-potassium transporting adenosine triphosphatase (ATP1A1), zonula occludens 1 (ZO-1), N-cadherin, N-CAM, and CD16630, 31, 32 (Figure S4). Furthermore, the cultured CECs displayed distinctive polygonal morphology.25, 33, 34 FISH was performed, using the same Cy3-(CAG)7 probe as described above, in three distinct primary FECD CEC lines and corresponding individual-matched fibroblast lines (Figure 2A). In each instance, bright nuclear foci were detected in the FECD CECs, similar to those previously identified in corneal tissue,15, 35 whereas the corresponding fibroblasts were foci negative, suggesting that the endothelial-specific context is important and that the cultured primary CECs represent an ideal ex vivo system to investigate CTG18.1 expansion-associated corneal endothelial pathology (Figure 2A)."}