Convergence of Human Genetics and Animal Studies: Gene Therapy for X-Linked Retinoschisis

Por Bush, Ronald A., Wei, Lisa L., Sieving, Paul A.
Publicado no Cold Spring Harb Perspect Med (2015)

Loss of Retinoschisin (RS1) Cell Surface Protein in Maturing Mouse Rod Photoreceptors Elevates the Luminance Threshold for Light-Driven Translocation of Transducin But Not Arrestin

Por Ziccardi, Lucia, Vijayasarathy, Camasamudram, Bush, Ronald A., Sieving, Paul A.
Publicado em 2012

Photoreceptor Pathology in the X-Linked Retinoschisis (XLRS) Mouse Results in Delayed Rod Maturation and Impaired Light Driven Transducin Translocation

Por Ziccardi, Lucia, Vijayasarathy, Camasamudram, Bush, Ronald A., Sieving, Paul A.
Publicado em 2014

Cone ERG Changes During Light Adaptation in Two All-Cone Mutant Mice: Implications for Rod-Cone Pathway Interactions

Por Bush, Ronald A., Tanikawa, Atsuhiro, Zeng, Yong, Sieving, Paul A.
Publicado no Invest Ophthalmol Vis Sci (2019)

Photoreceptor Protection by Adeno-Associated Virus–Mediated LEDGF Expression in the RCS Rat Model of Retinal Degeneration: Probing the Mechanism

Por Raz-Prag, Dorit, Zeng, Yong, Sieving, Paul A., Bush, Ronald A.
Publicado em 2009

Synaptic Pathology in Retinoschisis Knockout (Rs1(−)(/y)) Mouse Retina and Modification by rAAV-Rs1 Gene Delivery

Por Takada, Yuichiro, Vijayasarathy, Camasamudram, Zeng, Yong, Kjellstrom, Sten, Bush, Ronald A., Sieving, Paul A.
Publicado em 2008

Rearing Light Intensity Affects Inner Retinal Pathology in a Mouse Model of X-Linked Retinoschisis but Does Not Alter Gene Therapy Outcome

Por Marangoni, Dario, Yong, Zeng, Kjellström, Sten, Vijayasarathy, Camasamudram, A. Sieving, Paul, Bush, Ronald A.
Publicado no Invest Ophthalmol Vis Sci (2017)

Trans-ocular Electric Current In Vivo Enhances AAV-Mediated Retinal Gene Transduction after Intravitreal Vector Administration

Por Song, Hongman, Bush, Ronald A., Zeng, Yong, Qian, Haohua, Wu, Zhijian, Sieving, Paul A.
Publicado no Mol Ther Methods Clin Dev (2018)

Identification and Characterization of Two Mature Isoforms of Retinoschisin in Murine Retina

Por Vijayasarathy, Camasamudram, Gawinowicz, Mary Ann, Zeng, Yong, Takada, Yuichiro, Bush, Ronald A., Sieving, Paul A.
Publicado em 2006

Transgenic Expression of Constitutively Active RAC1 Disrupts Mouse Rod Morphogenesis

Por Song, Hongman, Bush, Ronald A., Vijayasarathy, Camasamudram, Fariss, Robert N., Kjellstrom, Sten, Sieving, Paul A.
Publicado em 2014

Intravitreal Ciliary Neurotrophic Factor Transiently Improves Cone-Mediated Function in a CNGB3(−/−) Mouse Model of Achromatopsia

Por Marangoni, Dario, Vijayasarathy, Camasamudram, Bush, Ronald A., Wei, Lisa L., Wen, Rong, Sieving, Paul A.
Publicado no Invest Ophthalmol Vis Sci (2015)

X-Linked Retinoschisis: RS1 Mutation Severity and Age Affect the ERG Phenotype in a Cohort of 68 Affected Male Subjects

Por Bowles, Kristen, Cukras, Catherine, Turriff, Amy, Sergeev, Yuri, Vitale, Susan, Bush, Ronald A., Sieving, Paul A.
Publicado em 2011

NADPH Oxidase Contributes to Photoreceptor Degeneration in Constitutively Active RAC1 Mice

Por Song, Hongman, Vijayasarathy, Camasamudram, Zeng, Yong, Marangoni, Dario, Bush, Ronald A., Wu, Zhijian, Sieving, Paul A.
Publicado no Invest Ophthalmol Vis Sci (2016)

Depleting Rac1 in mouse rod photoreceptors protects them from photo-oxidative stress without affecting their structure or function

Por Haruta, Masatoshi, Bush, Ronald A., Kjellstrom, Sten, Vijayasarathy, Camasamudram, Zeng, Yong, Le, Yun-Zheng, Sieving, Paul A.
Publicado em 2009

Inhibition of the visual cycle in vivo by 13-cis retinoic acid protects from light damage and provides a mechanism for night blindness in isotretinoin therapy

Por Sieving, Paul A., Chaudhry, Prem, Kondo, Mineo, Provenzano, Matthew, Wu, David, Carlson, Thomas J., Bush, Ronald A., Thompson, Debra A.
Publicado em 2001

Ciliary neurotrophic factor (CNTF) for human retinal degeneration: Phase I trial of CNTF delivered by encapsulated cell intraocular implants

Por Sieving, Paul A., Caruso, Rafael C., Tao, Weng, Coleman, Hanna R., Thompson, Darby J. S., Fullmer, Keri R., Bush, Ronald A.
Publicado em 2006

Loss of Circadian Photoentrainment and Abnormal Retinal Electrophysiology in Math5 Mutant Mice

Por Brzezinski, Joseph A., Brown, Nadean L., Tanikawa, Atsuhiro, Bush, Ronald A., Sieving, Paul A., Vitaterna, Martha H., Takahashi, Joseph S., Glaser, Tom
Publicado em 2005

Retinal Structure and Gene Therapy Outcome in Retinoschisin-Deficient Mice Assessed by Spectral-Domain Optical Coherence Tomography

Por Zeng, Yong, Petralia, Ronald S., Vijayasarathy, Camasamudram, Wu, Zhijian, Hiriyanna, Suja, Song, Hongman, Wang, Ya-Xian, Sieving, Paul A., Bush, Ronald A.
Publicado no Invest Ophthalmol Vis Sci (2016)

Probing potassium channel function in vivo by intracellular delivery of antibodies in a rat model of retinal neurodegeneration

Por Raz-Prag, Dorit, Grimes, William N., Fariss, Robert N., Vijayasarathy, Camasamudram, Campos, Maria M., Bush, Ronald A., Diamond, Jeffrey S., Sieving, Paul A.
Publicado em 2010

Preclinical Dose-Escalation Study of Intravitreal AAV-RS1 Gene Therapy in a Mouse Model of X-linked Retinoschisis: Dose-Dependent Expression and Improved Retinal Structure and Func...

Por Bush, Ronald A., Zeng, Yong, Colosi, Peter, Kjellstrom, Sten, Hiriyanna, Suja, Vijayasarathy, Camasamudram, Santos, Maria, Li, Jinbo, Wu, Zhijian, Sieving, Paul A.
Publicado no Hum Gene Ther (2016)