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XB-ART-56586
Dev Dyn May 1, 2020; 249 (5): 610-621.

Modeling ocular lens disease in Xenopus.

Viet J , Reboutier D , Hardy S , Lachke SA , Paillard L , Gautier-Courteille C .


Abstract
BACKGROUND: Ocular lens clouding is termed as cataract, which depending on the onset, is classified as congenital or age-related. Developing new cataract treatments requires new models. Thus far, Xenopus embryos have not been evaluated as a system for studying cataract. RESULTS: We characterized the developmental process of lens formation in Xenopus laevis tailbuds and tadpoles, and we disrupted the orthologues of three mammalian cataract-linked genes in F0 by CRISPR/Cas9. We assessed the consequences of gene inactivation by combining external examination with histochemical analyses and functional vision assays. Inactivating the key metazoan eye development transcription factor gene pax6 produces a strong eye phenotype including an absence of eye tissue. Inactivating the genes for gap-junction protein and a nuclease, gja8 and dnase2b, produces lens defects that share several features of human cataracts, including impaired vision acuity, nuclei retention in lens fiber cells, and actin fibers disorganization. We tested the potential improvement of the visual acuity of gja8 crispant tadpoles upon treatment with the molecular chaperone 4-phenylbutyrate. CONCLUSION: Xenopus is a valuable model organism to understand the molecular pathology of congenital eye defects, including cataracts, and to screen molecules with a potential to prevent or reverse cataracts.

PubMed ID: 31872467
Article link: Dev Dyn
Grant support: [+]
Genes referenced: dnase2b gja8 pam pax6
GO keywords: actin cytoskeleton organization [+]

Disease Ontology terms: coloboma [+]

Article Images: [+] show captions


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