PUBLICATION

Loss of Gap Junction Delta-2 (GJD2) gene orthologs leads to refractive error in zebrafish

Authors
Quint, W.H., Tadema, K.C.D., de Vrieze, E., Lukowicz, R.M., Broekman, S., Winkelman, B.H.J., Hoevenaars, M., de Gruiter, H.M., van Wijk, E., Schaeffel, F., Meester-Smoor, M., Miller, A.C., Willemsen, R., Klaver, C.C.W., Iglesias, A.I.
ID
ZDB-PUB-210605-2
Date
2021
Source
Communications biology   4: 676 (Journal)
Registered Authors
de Vrieze, Erik, Iglesias, Adriana, Miller, Adam
Keywords
none
MeSH Terms
  • Animals
  • Cataract/genetics
  • Connexins/genetics*
  • Connexins/metabolism
  • Disease Models, Animal*
  • Eye Proteins/genetics*
  • Eye Proteins/metabolism
  • Gene Expression Profiling/methods
  • Humans
  • Mutation*
  • Myopia/genetics
  • RNA-Seq/methods
  • Refractive Errors/genetics*
  • Retina/metabolism
  • Retina/pathology
  • Retinal Pigment Epithelium/metabolism
  • Retinal Pigment Epithelium/pathology
  • Single-Cell Analysis/methods
  • Zebrafish/genetics*
  • Zebrafish/metabolism
  • Zebrafish Proteins/genetics*
  • Zebrafish Proteins/metabolism
PubMed
34083742 Full text @ Commun Biol
Abstract
Myopia is the most common developmental disorder of juvenile eyes, and it has become an increasing cause of severe visual impairment. The GJD2 locus has been consistently associated with myopia in multiple independent genome-wide association studies. However, despite the strong genetic evidence, little is known about the functional role of GJD2 in refractive error development. Here, we find that depletion of gjd2a (Cx35.5) or gjd2b (Cx35.1) orthologs in zebrafish, cause changes in the biometry and refractive status of the eye. Our immunohistological and scRNA sequencing studies show that Cx35.5 (gjd2a) is a retinal connexin and its depletion leads to hyperopia and electrophysiological changes in the retina. These findings support a role for Cx35.5 (gjd2a) in the regulation of ocular biometry. Cx35.1 (gjd2b) has previously been identified in the retina, however, we found an additional lenticular role. Lack of Cx35.1 (gjd2b) led to a nuclear cataract that triggered axial elongation. Our results provide functional evidence of a link between gjd2 and refractive error.
Genes / Markers
Figures
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Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping