Zobrazeno 1 - 10
of 66
pro vyhledávání: '"J Hugh McDowell"'
Publikováno v:
PLoS ONE, Vol 11, Iss 2, p e0148773 (2016)
PURPOSE:Bardet-Biedl syndrome is a complex ciliopathy that usually manifests with some form of retinal degeneration, amongst other ciliary-related deficiencies. One of the genetic causes of this syndrome results from a defect in Bardet-Biedl Syndrome
Externí odkaz:
https://doaj.org/article/1a739a90ec1145d5a8331fdc54dbaad3
Publikováno v:
BioTechniques, Vol 21, Iss 5, Pp 788-792 (1996)
Externí odkaz:
https://doaj.org/article/9b6e379eb5e04674962127fdaacb7220
Autor:
Lei Lei, J. Hugh McDowell, Shibo Tang, W. Clay Smith, Huapeng Li, Shalesh Kaushal, Radouil Tzekov, Guangping Gao
Publikováno v:
International Journal of Molecular Sciences
International Journal of Molecular Sciences; Volume 18; Issue 4; Pages: 728
International Journal of Molecular Sciences, Vol 18, Iss 4, p 728 (2017)
International Journal of Molecular Sciences; Volume 18; Issue 4; Pages: 728
International Journal of Molecular Sciences, Vol 18, Iss 4, p 728 (2017)
The accumulation of lipofuscin in the retinal pigment epithelium (RPE) is dependent on the effectiveness of photoreceptor outer segment material degradation. This study explored the role of autophagy in the fate of RPE lipofuscin degradation. After s
Autor:
Robert B. Nussenblatt, Eddie A. James, Rachel R. Caspi, J. Hugh McDowell, H. Nida Sen, Reiko Horai, Chi-Chao Chan, Zaruhi Karabekian, Chella S. David, Phyllis B. Silver, William W. Kwok, Mary J. Mattapallil, Joseph J. Mattapallil
Publikováno v:
The Journal of Immunology. 187:1977-1985
Noninfectious uveitis is a leading cause of blindness and thought to involve autoimmune T cell responses to retinal proteins (e.g., retinal arrestin [soluble-Ag (S-Ag)]). There are no known biomarkers for the disease. Susceptibility is associated wit
Publikováno v:
Journal of Biological Chemistry. 282:25560-25568
In this study we investigate conformational changes in Loop V-VI of visual arrestin during binding to light-activated, phosphorylated rhodopsin (Rho*-P) using a combination of site-specific cysteine mutagenesis and intramolecular fluorescence quenchi
Autor:
Thomas C. Foster, Qiuhong Li, Jijing Pang, Steven Nusinowitz, Shalesh Kaushal, Huashi Li, William W. Hauswirth, Vince A. Chiodo, Jie Li, Bo Chang, Ritu Malhotra, Asha Rani, Jacqueline T. Teusner, J. Hugh McDowell, Seok-Hong Min, Thomas J. Doyle, Syed Mohammed Noorwez, Ashok Kumar
Publikováno v:
Molecular Therapy. 13(3):565-572
Retinal pigment epithelium-specific protein 65 kDa (RPE65) is a protein responsible for isomerization of all-trans-retinaldehyde to its photoactive 11-cis-retinaldehyde and is essential for the visual cycle. RPE65 mutations can cause severe, early on
Autor:
Paul A. Hargrave, Patricia C. Edwards, Manfred Burghammer, Gebhard F. X. Schertler, J. Hugh McDowell, Jade Li, Claudio Villa
Publikováno v:
Journal of Molecular Biology. 343:1439-1450
Rhodopsin, the pigment protein responsible for dim-light vision, is a G protein-coupled receptor that converts light absorption into the activation of a G protein, transducin, to initiate the visual response. We have crystallised detergent-solubilise
Autor:
Syed Mohammed Noorwez, Karen Smith, Mark P. Krebs, Ritu Malhotra, J. Hugh McDowell, Shalesh Kaushal
Publikováno v:
Journal of Biological Chemistry. 279:16278-16284
The clinically common mutant opsin P23H, associated with autosomal dominant retinitis pigmentosa, yields low levels of rhodopsin when retinal is added following induction of the protein in stably transfected HEK-293 cells. We previously showed that P
Publikováno v:
FEBS Letters. 564:307-311
Visual arrestin binds to the phosphorylated carboxy-terminal region of rhodopsin to block interactions with transducin and terminate signaling in the rod photoreceptor cells. A synthetic seven-phospho-peptide from the C-terminal region of rhodopsin,
Autor:
J. Hugh McDowell, Paul A. Hargrave, W. Clay Smith, Giuseppina Pennesi, Zaruhi Karabekian, Chella S. David, Mary J. Mattapallil, Larry A. Donoso, Phyllis B. Silver, Barbara Wiggert, Shu Hui Sun, Dody Avichezer, Chi-Chao Chan, Rachel R. Caspi
Publikováno v:
Journal of Clinical Investigation. 111:1171-1180
Experimental autoimmune uveitis (EAU) is a disease of the neural retina induced by immunization with retinal antigens, such as interphotoreceptor retinoid-binding protein (IRBP) and arrestin (retinal soluble antigen, S-Ag). EAU serves as a model for