In:
Frontiers in Cell and Developmental Biology, Frontiers Media SA, Vol. 9 ( 2021-2-18)
Kurzfassung:
Primary cilia are sensory organelles vital for developmental and physiological processes. Their dysfunction causes a range of phenotypes including retinopathies. Although primary cilia have been described in the retinal pigment epithelium (RPE), little is known about their contribution to biological processes within this tissue. Ciliary proteins are increasingly being identified in non-ciliary locations and might carry out additional functions, disruption of which possibly contributes to pathology. The RPE is essential for maintaining photoreceptor cells and visual function. We demonstrate that upon loss of Bbs8 , predominantly thought to be a ciliary gene, the RPE shows changes in gene and protein expression initially involved in signaling pathways and developmental processes, and at a later time point RPE homeostasis and function. Differentially regulated molecules affecting the cytoskeleton and cellular adhesion, led to defective cellular polarization and morphology associated with a possible epithelial-to-mesenchymal transition (EMT)-like phenotype. Our data highlights the benefit of combinatorial “omics” approaches with in vivo data for investigating the function of ciliopathy proteins. It also emphasizes the importance of ciliary proteins in the RPE and their contribution to visual disorders, which must be considered when designing treatment strategies for retinal degeneration.
Materialart:
Online-Ressource
ISSN:
2296-634X
DOI:
10.3389/fcell.2021.607121
DOI:
10.3389/fcell.2021.607121.s001
DOI:
10.3389/fcell.2021.607121.s002
DOI:
10.3389/fcell.2021.607121.s003
DOI:
10.3389/fcell.2021.607121.s004
DOI:
10.3389/fcell.2021.607121.s005
DOI:
10.3389/fcell.2021.607121.s006
DOI:
10.3389/fcell.2021.607121.s007
DOI:
10.3389/fcell.2021.607121.s008
DOI:
10.3389/fcell.2021.607121.s009
DOI:
10.3389/fcell.2021.607121.s010
DOI:
10.3389/fcell.2021.607121.s011
DOI:
10.3389/fcell.2021.607121.s012
DOI:
10.3389/fcell.2021.607121.s013
Sprache:
Unbekannt
Verlag:
Frontiers Media SA
Publikationsdatum:
2021
ZDB Id:
2737824-X
