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Spata7 is required for maintenance of the retinal connecting cilium
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Journal Article
Spata7 is required for maintenance of the retinal connecting cilium
2022
Overview
SPATA7
, an early onset LCA3 retinal disease gene, encodes a putative scaffold protein that is essential for the proper assembly of the connecting cilium (CC) complex in photoreceptors. Previous studies have shown that SPATA7 interacts with other photoreceptor-specific ciliary proteins, such as RPGR and RPGRIP1, and maintains the integrity of CC integrity. However, although it is known that
Spata7
is required for early formation of the CC, it is unclear if Spata7 is also required for the maintenance of the CC. To investigate
Spata7
function in the retina at the adult stage, loss of function was induced in the adult retina upon tamoxifen induction of an inducible
Spata7
knockout allele (
Spata7
flox/−
;
UbcCreERT2/
+
). The phenotype of mutant retina was characterized by a combination of histology, immunobiochemistry, and electroretinography (ERG). Our results demonstrated that
Spata7
is also essential for maintaining the integrity of the mature retinal CC. Loss of
Spata7
in adults caused phenotypes similar to those seen in germline mutant mice, including photoreceptor cell degeneration and defective ERG responses. Close examination of the CC revealed significantly shortened NPHP1 length as a result of
Spata7
deletion. Furthermore, mislocalization of rhodopsin, leading to ER stress-mediated apoptosis, was observed in the retinal layers. Our results indicate that
Spata7
is required not only for the establishment but also for the maintenance of the CC of photoreceptors.
Publisher
Nature Publishing Group UK,Nature Publishing Group,Nature Portfolio
Subject
