Zobrazeno 1 - 10
of 265
pro vyhledávání: '"Paul A. Sieving"'
Autor:
Yong Zeng, Shasha Gao, Yichao Li, Dario Marangoni, Tharindu De Silva, Wai T. Wong, Emily Y. Chew, Xun Sun, Tiansen Li, Paul A. Sieving, Haohua Qian
Publikováno v:
Bioengineering, Vol 11, Iss 5, p 449 (2024)
Optical coherence tomography (OCT) is widely used to probe retinal structure and function. This study investigated the outer retina band (ORB) pattern and reflective intensity for the region between bands 2 and 3 (Dip) in three mouse models of inheri
Externí odkaz:
https://doaj.org/article/f03fe0017b6b4d25b49be8d7ecd9e453
Autor:
Kiyoharu J. Miyagishima, Ruchi Sharma, Malika Nimmagadda, Katharina Clore-Gronenborn, Zoya Qureshy, Davide Ortolan, Devika Bose, Mitra Farnoodian, Congxiao Zhang, Andrew Fausey, Yuri V. Sergeev, Mones Abu-Asab, Bokkyoo Jun, Khanh V. Do, Marie-Audrey Kautzman Guerin, Jorgelina Calandria, Aman George, Bin Guan, Qin Wan, Rachel C. Sharp, Catherine Cukras, Paul A. Sieving, Robert B. Hufnagel, Nicolas G. Bazan, Kathleen Boesze-Battaglia, Sheldon Miller, Kapil Bharti
Publikováno v:
Communications Biology, Vol 4, Iss 1, Pp 1-16 (2021)
Miyagishima et al. investigate the pathological mechanisms underlying mutant CTRP5 function in late-onset retinal degeneration (L-ORD). With human iPSC-derived RPE cells, they demonstrate that in L-ORD iRPE, constitutive activation of AMPK disrupts c
Externí odkaz:
https://doaj.org/article/24727da70edd48558b39804aab74c898
Autor:
Yong Zeng, Ryan Boyd, Joshua Bartoe, Henry E. Wiley, Dario Marangoni, Lisa L. Wei, Paul A. Sieving
Publikováno v:
Molecular Therapy: Methods & Clinical Development, Vol 18, Iss , Pp 422-427 (2020)
Intravitreal administration for human adeno-associated vector (AAV) delivery is easier and less traumatic to ocular tissues than subretinal injection, but it gives limited retinal transduction. AAV vectors are large (about 4,000 kDa) compared with mo
Externí odkaz:
https://doaj.org/article/81bf04d9c7754b298b561135526c4165
Publikováno v:
Genes, Vol 13, Iss 11, p 1995 (2022)
We generated a Long Evans transgenic rat with targeted deletion of the whole Rs1 exon-1 and evaluated the pathological retinal phenotype of this Rs1-/Y rat model of X-linked retinoschisis (XLRS). The Rs1−/Y rat exhibited very early onset and rapidl
Externí odkaz:
https://doaj.org/article/711f39e4245941b8955dea59e36d4976
Publikováno v:
Molecular Therapy: Methods & Clinical Development, Vol 13, Iss , Pp 77-85 (2019)
Adeno-associated virus (AAV) vector-mediated gene delivery is a promising approach for therapy, but implementation in the eye currently is hampered by the need for delivering the vector underneath the retina, using surgical application into the subre
Externí odkaz:
https://doaj.org/article/2704653eb2ce4825a34dbb7d049e31be
Autor:
Chelsea Bender, Elizabeth Geena Woo, Bin Guan, Ehsan Ullah, Eric Feng, Amy Turriff, Santa J. Tumminia, Paul A. Sieving, Catherine A. Cukras, Robert B. Hufnagel
Publikováno v:
Genes, Vol 13, Iss 4, p 675 (2022)
For disorders with X-linked inheritance, variants may be transmitted through multiple generations of carrier females before an affected male is ascertained. Pathogenic RS1 variants exclusively cause X-linked retinoschisis (XLRS). While RS1 is constra
Externí odkaz:
https://doaj.org/article/b4f9c04f070c44cbaa1425398e99dbc0
Publikováno v:
Disease Models & Mechanisms, Vol 8, Iss 2, Pp 109-129 (2015)
Retinal neurodegeneration associated with the dysfunction or death of photoreceptors is a major cause of incurable vision loss. Tremendous progress has been made over the last two decades in discovering genes and genetic defects that lead to retinal
Externí odkaz:
https://doaj.org/article/226bb49833144d8f8214a118cf1e1e8b
Autor:
Camiel J. F. Boon, Julie De Zaeytijd, Mary J. van Schooneveld, Maria M. van Genderen, Ralph J. Florijn, Bart P. Leroy, Carel B. Hoyng, Paul A. Sieving, Alberta A H J Thiadens, Sophie Walraedt, Jeannette Ossewaarde-van Norel, Magda A. Meester-Smoor, Leo C. Hahn, Birgit I. Lissenberg-Witte, Arthur A.B. Bergen, Jacoline B. ten Brink, Elfride De Baere, Nieneke L. Wesseling, Caroline Van Cauwenbergh, Caroline C W Klaver, Roselie M. Diederen, L. Ingeborgh van den Born, Ine Strubbe
Publikováno v:
Ophthalmology. 129:191-202
Purpose To describe the natural course, phenotype and genotype of patients with X-linked retinoschisis (XLRS). Design Retrospective cohort study. Participants Three hundred forty patients with XLRS from 178 presumably unrelated families. Methods This
Autor:
Tzu-Ni Sin, Sangbae Kim, Yumei Li, Jun Wang, Rui Chen, Sook Hyun Chung, Soohyun Kim, M. Isabel Casanova, Sangwan Park, Zeljka Smit-McBride, Ning Sun, Ori Pomerantz, Jeffrey A. Roberts, Bin Guan, Robert B. Hufnagel, Ala Moshiri, Sara M. Thomasy, Paul A. Sieving, Glenn Yiu
Publikováno v:
Investigative ophthalmology & visual science, vol 64, iss 1
PurposeFoveoschisis involves the pathologic splitting of retinal layers at the fovea, which may occur congenitally in X-linked retinoschisis (XLRS) or as an acquired complication of myopia. XLRS is attributed to functional loss of the retinal adhesio
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::09bd4ac56d94203667e046faa22a1dd4
https://escholarship.org/uc/item/3xz196xw
https://escholarship.org/uc/item/3xz196xw
Autor:
Haohua Qian, Paul A. Sieving, Camasamudram Vijayasarathy, Yichao Li, Maria M Campos, Yong Zeng
Publikováno v:
Gene Therapy. 29:431-440
Animal models of X-linked juvenile retinoschisis (XLRS) are valuable tools for understanding basic biochemical function of retinoschisin (RS1) protein and to investigate outcomes of preclinical efficacy and toxicity studies. In order to work with an