https://transhumanist.ru/index.php?action=history&feed=atom&title=CNGB3 2026-06-03T21:36:22Z История изменений этой страницы в вики MediaWiki 1.43.6 https://transhumanist.ru/index.php?title=CNGB3&diff=5321&oldid=prev 2021-05-12T13:39:03Z

<p>Новая страница: «Cyclic nucleotide-gated cation channel beta-3 (Cone photoreceptor cGMP-gated channel subunit beta) (Cyclic nucleotide-gated cation channel modulatory subunit) (Cy...»</p> <p><b>Новая страница</b></p><div>Cyclic nucleotide-gated cation channel beta-3 (Cone photoreceptor cGMP-gated channel subunit beta) (Cyclic nucleotide-gated cation channel modulatory subunit) (Cyclic nucleotide-gated channel beta-3) (CNG channel beta-3)<br /> <br /> ==Publications==<br /> <br /> {{medline-entry<br /> |title=Long-term and age-dependent restoration of visual function in a mouse model of [[CNGB3]]-associated achromatopsia following gene therapy.<br /> |pubmed-url=https://pubmed.ncbi.nlm.nih.gov/21576125<br /> |abstract=Mutations in the [[CNGB3]] gene account for &gt;50% of all known cases of achromatopsia. Although of early onset, its stationary character and the potential for rapid assessment of restoration of retinal function following therapy renders achromatopsia a very attractive candidate for gene therapy. Here we tested the efficacy of an rAAV2/8 vector containing a human cone arrestin promoter and a human [[CNGB3]] cDNA in [[CNGB3]] deficient mice. Following subretinal delivery of the vector, [[CNGB3]] was detected in both M- and S-cones and resulted in increased levels of [[CNGA3]], increased cone density and survival, improved cone outer segment structure and normal subcellular compartmentalization of cone opsins. Therapy also resulted in long-term improvement of retinal function, with restoration of cone [[ERG]] amplitudes of up to 90% of wild-type and a significant improvement in visual acuity. Remarkably, successful restoration of cone function was observed even when treatment was initiated at 6 months of age; however, restoration of normal visual acuity was only possible in younger animals (e.g. 2-4 weeks old). This study represents achievement of the most substantial restoration of visual function reported to date in an animal model of achromatopsia using a human gene construct, which has the potential to be utilized in clinical trials.<br /> |mesh-terms=* Aging<br /> * Animals<br /> * Arrestins<br /> * Cell Survival<br /> * Color Vision Defects<br /> * Cyclic Nucleotide-Gated Cation Channels<br /> * Disease Models, Animal<br /> * Gene Transfer Techniques<br /> * Genetic Therapy<br /> * Genetic Vectors<br /> * Humans<br /> * Injections<br /> * Mice<br /> * Mice, Transgenic<br /> * Opsins<br /> * Organ Specificity<br /> * Promoter Regions, Genetic<br /> * Protein Transport<br /> * Retina<br /> * Retinal Cone Photoreceptor Cells<br /> * Time Factors<br /> * Vision, Ocular<br /> * Visual Acuity<br /> <br /> |full-text-url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3140821<br /> }}</div>

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