Editorial
doi: 10.1001/archophthalmol.2010.245.
Blind dogs that can see: pharmacological treatment of Leber congenital amaurosis caused by a defective visual cycle
- PMID: 21060052
- PMCID: PMC4495581
- DOI: 10.1001/archophthalmol.2010.245
Item in Clipboard
Editorial
Blind dogs that can see: pharmacological treatment of Leber congenital amaurosis caused by a defective visual cycle
Arch Ophthalmol.
2010 Nov.
No abstract available
Figures
Treatment of Briard Rpe65−/−-mutant
dogs with the synthetic cis-retinoids. A, View of a treated
Briard Rpe65−/−-mutant dog. B,
Chemistry of vision: phototransduction and the retinoid (visual) cycle (reviewed
in von Lintig et al).
Mutations in Rpe65 are associated with Leber congenital
amaurosis because they
are required for 11-cis-retinal production.
9-cis-Retinoids can be used to overcome this blockage and
regenerate functional visual pigments, called isorhodopsin, in
rods that have characteristics similar to rhodopsin. The depicted protein
structures are of rhodopsin (left) and Rpe65 (right).
Comment on
-
Improvement of visual performance with intravitreal administration of 9-cis-retinal in Rpe65-mutant dogs.Arch Ophthalmol. 2010 Nov;128(11):1442-8. doi: 10.1001/archophthalmol.2010.210. Epub 2010 Sep 13. Arch Ophthalmol. 2010. PMID: 20837787
Similar articles
-
Improvement of visual performance with intravitreal administration of 9-cis-retinal in Rpe65-mutant dogs.Arch Ophthalmol. 2010 Nov;128(11):1442-8. doi: 10.1001/archophthalmol.2010.210. Epub 2010 Sep 13. Arch Ophthalmol. 2010. PMID: 20837787
-
Effect of an intravitreal antisense oligonucleotide on vision in Leber congenital amaurosis due to a photoreceptor cilium defect.Nat Med. 2019 Feb;25(2):225-228. doi: 10.1038/s41591-018-0295-0. Epub 2018 Dec 17. Nat Med. 2019. PMID: 30559420 Clinical Trial.
-
Inverse correlation between fatty acid transport protein 4 and vision in Leber congenital amaurosis associated with RPE65 mutation.Proc Natl Acad Sci U S A. 2020 Dec 15;117(50):32114-32123. doi: 10.1073/pnas.2012623117. Epub 2020 Nov 30. Proc Natl Acad Sci U S A. 2020. PMID: 33257550 Free PMC article.
-
Leber Congenital Amaurosis (LCA): Potential for Improvement of Vision.Invest Ophthalmol Vis Sci. 2019 Apr 1;60(5):1680-1695. doi: 10.1167/iovs.19-26672. Invest Ophthalmol Vis Sci. 2019. PMID: 31009524 Free PMC article. Review. No abstract available.
-
Leber Congenital Amaurosis.Adv Exp Med Biol. 2018;1085:131-137. doi: 10.1007/978-3-319-95046-4_26. Adv Exp Med Biol. 2018. PMID: 30578499 Review.
Cited by
-
QLT091001, a 9-cis-retinal analog, is well-tolerated by retinas of mice with impaired visual cycles.Invest Ophthalmol Vis Sci. 2013 Jan 17;54(1):455-66. doi: 10.1167/iovs.12-11152. Invest Ophthalmol Vis Sci. 2013. PMID: 23249702 Free PMC article.
-
A microparticle/hydrogel combination drug-delivery system for sustained release of retinoids.Invest Ophthalmol Vis Sci. 2012 Sep 19;53(10):6314-23. doi: 10.1167/iovs.12-10279. Invest Ophthalmol Vis Sci. 2012. PMID: 22918645 Free PMC article.
-
Chemistry and biology of the initial steps in vision: the Friedenwald lecture.Invest Ophthalmol Vis Sci. 2014 Oct 22;55(10):6651-72. doi: 10.1167/iovs.14-15502. Invest Ophthalmol Vis Sci. 2014. PMID: 25338686 Free PMC article.
-
Chemistry and biology of vision.J Biol Chem. 2012 Jan 13;287(3):1612-9. doi: 10.1074/jbc.R111.301150. Epub 2011 Nov 10. J Biol Chem. 2012. PMID: 22074921 Free PMC article. Review.
References
-
- Gearhart PM, Gearhart G, Thompson DA, Petersen-Jones SM. Improvement of visual performance with intravitreal administration of 9-cis-retinal in Rpe65-mutant dogs. Arch Ophthalmol. 2010;128(11):1442–1448. - PubMed
-
- Baker H, Schor SM, Murphy BD, DeAngelis B, Feingold S, Frank O. Blood vitamin and choline concentrations in healthy domestic cats, dogs, and horses. Am J Vet Res. 1986;47(7):1468–1471. - PubMed
