This paper (and the cover article) is the result of a collaborative effort between Damian C. Lee, Felix R. Vazquez-Chona, W. Drew Ferrell, Beatrice M. Tam, Bryan W. Jones, Robert E. Marc, and Orson L. Moritz.
This paper in PNAS by William A. Beltran, Artur V. Cideciyan, Alfred S. Lewin, Simone Iwabe, Hemant Khanna, Alexander Sumaroka, Vince A. Chiodo, Diego S. Fajardo, Alejandro J. Román, Wen-Tao Deng, Malgorzata Swider, Tomas S. Alemán, Sanford L. Boye, Sem Genini, Anand Swaroop, William W. Hauswirth, Samuel G. Jacobson and Gustavo D. Aguirre is a continuation of their work in retinal degeneration, this form of retinal degeneration, X-linked retinitis pigmentosa (RP).
Hereditary retinal dystrophies (retinitis pigmentosa, Leber congenital amaurosis, cone-rod dystrophies, macular degeneration) are characterized by loss of visual function, sometimes starting during early childhood, other times in late adulthood. About 30% of these dystrophies are inherited in an autosomal dominant fashion (RetNet), caused by gain-of-function mutant alleles which encode a malignant form of a normal protein. Continue reading “Notable Paper: Long-term RNA interference gene therapy in a dominant retinitis pigmentosa mouse model”
This paper by Vazquez-Chona FR, Swan A, Ferrell WD, Jiang L, Baehr W, Chien WM, Fero M, Marc RE and Levine EM addresses a long standing issue in the field of neuroscience: is the reactive phenotype of glial cells in and of itself detrimental to neural survival or function?
This paper by Y Lin, BW Jones, A Liu, JF Tucker, K Rapp, L Luo, W Baehr, PS Bernstein, CB Watt, JH Yang, MV Shaw and RE Marc examines the neuronal sprouting or neuritogenesis components of retinal remodeling found in retinal degenerative disease and describes a control process for retinoid X receptors (RXRs) in neuritogenesis. Continue reading “Retinoid Receptors Trigger Neuritogenesis in Retinal Degenerations”
This paper is the result of a collaborative effort between Bryan William Jones, Mineo Kondo and Hiroko Terasaki, Carl Watt, Kevin Rapp, James Anderson, Yanhua Lin, Maggie Shaw, Jia-Hui Yang and Robert Marc.
This work presents a substantial advance in models of Retinitis pigmentosa (RP), an set of inherited blinding diseases characterized by progressive loss of retinal photoreceptors. Continue reading “Notable Paper: Retinal Remodeling in the Tg P347L Rabbit, a Large-Eye Model of Retinal Degeneration”
This truly groundbreaking paper by M Mehdi Doroudchi, Kenneth P Greenberg, Jianwen Liu, Kimberly A Silka, Edward S Boyden, Jennifer A Lockridge, A Cyrus Arman, Ramesh Janani, Shannon E Boye, Sanford L Boye, Gabriel M Gordon, Benjamin C Matteo, Alapakkam P Sampath, William W Hauswirth and Alan Horsager demonstrates that channelrhodopsin-2, a cation channel from algae than can be gated/activated by light can restore both physiological and behavioral visual responses in mice with retinal degenerative disease.
It is been long known that ventral and central cones degenerate more rapidly than dorsal cones in retinoid-deprived LCA (Leber’s Congenital Amaurosis) models. This paper in PNAS by Tao Zhang, Wolfgang Baehr…