What is it about?
Peroxisomes are small subcellular organelles that carry out various important functions ranging from fatty acid oxidation to antioxidant defence. Patients with peroxisomal disorders often suffer from retinal defects and loss of vision. However, the cause for this retinal degeneration remains elusive. Moreover, the relative importance of these organelles to the various cell-types of the retina is also unknown. Using mice models, we show that peroxisomal fatty acid oxidation is crucial for the proper functioning of the retinal pigment epithelium (RPE), an important monolayer of cells that constitutes an integral part of the blood-retinal barrier. A loss of this function in the RPE cells leads to RPE dysfunction and a secondary degeneration of the underlying cells of the retina.
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Why is it important?
Our findings shed light onto the role of peroxisomes in the retinal pigment epithelium (RPE) cells, shifting the importance of these organelles from photoreceptors to the RPE. They also provide clues as to the cause and origins of retinal degeneration observed in peroxisomal disorder patients, and warn that the RPE must be targeted in addition to the photoreceptors in therapeutic strategies for such patients.
Perspectives
At the start of this work, I was surprised to be made aware that we did not know the role played by peroxisomes in the retina, especially considering the fact that patients with peroxisomal disorders often suffer from retinal degeneration. I am excited that the knowledge created from this study significantly advances our understanding of the retina and helps develop appropriate treatment strategies that would help patients.
Sai Kocherlakota
Associatie KU Leuven
Read the Original
This page is a summary of: The murine retinal pigment epithelium requires peroxisomal β-oxidation to maintain lysosomal function and prevent dedifferentiation, Proceedings of the National Academy of Sciences, October 2023, Proceedings of the National Academy of Sciences,
DOI: 10.1073/pnas.2301733120.
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