PUBLICATION

The Role of TRiC-enhanced Actin Folding in Leber Congenital Amaurosis

Authors
Berger, S., D Currie, P., Berger, J.
ID
ZDB-PUB-230321-37
Date
2023
Source
Journal of Ophthalmic & Vision Research   18: 606760-67 (Journal)
Registered Authors
Berger, Joachim, Berger, Silke
Keywords
Leber Congenital Amaurosis, Retina, TRiC, Zebrafish, cct5, Actin
MeSH Terms
none
PubMed
36937198 Full text @ J. Ophthalmic Vis. Res.
Citation
Berger, S., D Currie, P., Berger, J. (2023) The Role of TRiC-enhanced Actin Folding in Leber Congenital Amaurosis. Journal of Ophthalmic & Vision Research. 18:606760-67.
Abstract
Purpose Mutations in TCP-1 ring complex (TRiC) have been associated with Leber Congenital Amaurosis (LCA). TRiC is involved in protein folding and has 8 essential subunits including CCT5. Herein, we studied the retina of TRiC mutant zebrafish to evaluate the possible role of impaired actin and tubulin folding in LCA.
Methods The cct5tf212b retina was histologically studied using Toluidine Blue staining as well as TUNEL, BrdU-labeling, and Phalloidin assays. Retinal organisation was assessed by quantification of the cellularity utilising DAPI.
Results Laminar organization of cct5tf212b retinas was intact. Enhanced apoptosis throughout the cct5tf212b retina was not compensated by higher proliferation rates, leaving the cct5tf212b retina smaller in size. Quantification of retinal layer cellularity demonstrated that specifically the numbers of the amacrine and the retinal ganglion cells were depleted, suggesting that the cct5tf212b retina was not uniformly affected by the reduced actin folding.
Conclusion Whereas the current literature suggests that LCA is predominantly affecting retinal photoreceptor cells and the retinal pigment epithelium, cct5tf212b analyses demonstrated the important role of folding of actin by TRiC, suggesting that cct5tf212b is a useful tool to specifically analyze the role of F-actin filaments in the context of LCA.
Genes / Markers
Figures
Show all Figures
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping
Your Input Welcome
We welcome your input and comments. Please use this form to recommend updates to the information in ZFIN. We appreciate as much detail as possible and references as appropriate. We will review your comments promptly.
Please check the highlighted fields and try again.
Thank you for submitting comments. Your input has been emailed to ZFIN curators who may contact you if additional information is required.
Oops. Something went wrong. Please try again later.