期刊论文

英文

Cell

0092-8674

2024

187

6

1387-1401.e13

10.1016/j.cell.2024.01.040

Acknowledgments We thank all members of the Wei Laboratories for their support and discussion. This work was supported by the National Key Research and Development Program of China 2021YFA1101200 (2021YFA1101204) to L.W., F.Z., and X.W. and by the National Key Research and Development Program of China 2021YFA0805904 to H.W. In addition, R.W.J.L. received funding from the National Institute for Health and Care Research (NIHR) Biomedical Research Centre at Moorfields Eye Hospital NHS Foundation Trust and UCL

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The Crumbs homolog 1 (CRB1) gene is associated with retinal degeneration, most commonly Leber congenital amaurosis (LCA) and retinitis pigmentosa (RP). Here, we demonstrate that murine retinas bearing the Rd8 mutation of Crb1 are characterized by the presence of intralesional bacteria. While normal CRB1 expression was enriched in the apical junctional complexes of retinal pigment epithelium and colonic enterocytes, Crb1 mutations dampened its expression at both sites. Consequent impairment of the outer blood retinal barrier and colonic intestinal epithelial barrier in Rd8 mice led to the trans...