Elimination of a Retinal Riboflavin Binding Protein Exacerbates Degeneration in a Model of Cone-Rod Dystrophy

Investigative Ophthalmology & Visual Science
Ayse M GencMuna I Naash

Abstract

Riboflavin and its cofactors are essential for cellular energy generation, responses to oxidative stress, and overall homeostasis. Retbindin is a novel retina-specific riboflavin binding protein essential for the maintenance of retinal flavin levels, but its function remains poorly understood. To further elucidate the function of retbindin in retinal health and disease, we evaluated its role in retinal degeneration in a cone-rod dystrophy model associated with the R172W mutation in the photoreceptor tetraspanin Prph2. We performed structural, functional, and biochemical characterization of R172W-Prph2 mice with and without retbindin (Rtbdn-/-/Prph2R172W). Retbindin is significantly upregulated during degeneration in the R172W model, suggesting that retbindin plays a protective role in retinal degenerative diseases. This hypothesis was supported by our findings that R172W mice lacking retbindin (Rtbdn-/-/Prph2R172W) exhibit functional and structural defects in rods and cones that are significantly worse than in controls. Retinal flavin levels were also altered in the Rtbdn-/-/Prph2R172W retina. However, in contrast to the Rtbdn-/- retina which has reduced flavin levels compared to wild-type, Rtbdn-/-/Prph2R172W retinas exhibited...Continue Reading

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Citations

Sep 29, 2020·Frontiers in Cell and Developmental Biology·Tirthankar SinhaMuayyad R Al-Ubaidi
Feb 3, 2021·Proceedings of the National Academy of Sciences of the United States of America·Tirthankar SinhaMuayyad R Al-Ubaidi
Apr 6, 2021·Frontiers in Neuroscience·Ryan CraneMuna I Naash

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Methods Mentioned

BETA
transgenic
genotyping
Assay

Software Mentioned

Airyscan
Bio
Zen 2 lite
Rad Image Lab

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