Zobrazeno 1 - 10
of 15
pro vyhledávání: '"O. V. Bulgakov"'
Autor:
Basil S. Pawlyk, Xiaoyun Xu, Xun Sun, Michael A. Sandberg, Michael Adamian, Shahrokh C. Khani, Xiao-Qing Liu, O. V. Bulgakov, Eliot L. Berson, Taibo Li
Publikováno v:
Human Gene Therapy. 21:993-1004
RPGR-interacting protein-1 (RPGRIP1) is localized in the photoreceptor-connecting cilium, where it anchors the RPGR (retinitis pigmentosa GTPase regulator) protein, and its function is essential for photoreceptor maintenance. Genetic defect in RPGRIP
Autor:
O. V. Bulgakov, Michael Adamian, Keith N. Darrow, M. Charles Liberman, Basil S. Pawlyk, Xiaoqing Liu, Tiansen Li
Publikováno v:
Proceedings of the National Academy of Sciences. 104:4413-4418
Usher syndrome type IIA (USH2A), characterized by progressive photoreceptor degeneration and congenital moderate hearing loss, is the most common subtype of Usher syndrome. In this article, we show that the USH2A protein, also known as usherin, is an
Publikováno v:
Developmental Biology. 290(1):1-12
Sonic hedgehog (Shh) signaling is required for the growth and patterning of many tissues in vertebrate embryos, but important aspects of the Shh signal transduction pathway are poorly understood. For example, the vesicle transport protein Rab23 is a
Autor:
Tiansen Li, Gordon L. Fain, Clint L. Makino, Xiao Hong Wen, Xiaoqing Liu, Jun Yang, Basil S. Pawlyk, Michael A. Sandberg, O. V. Bulgakov, Michael L. Woodruff
Publikováno v:
Proceedings of the National Academy of Sciences. 101:13903-13908
Aryl hydrocarbon receptor-interacting protein-like 1 (AIPL1) is a member of the FK-506-binding protein family expressed specifically in retinal photoreceptors. Mutations in AIPL1 cause Leber congenital amaurosis, a severe early-onset retinopathy that
Publikováno v:
Development. 131:2149-2159
Sonic hedgehog (SHH) is a secreted morphogen that regulates the patterning and growth of many tissues in the developing mouse embryo, including the central nervous system (CNS). We show that a member of the FK506-binding protein family, FKBP8, is an
Publikováno v:
The Journal of Cell Biology
The ciliary rootlet, first recognized over a century ago, is a prominent structure originating from the basal body at the proximal end of a cilium. Despite being the largest cytoskeleton, its structural composition has remained unknown. Here, we repo
Autor:
O. V. Bulgakov, Alexander J. Smith, Basil S. Pawlyk, Xun Sun, Robin R. Ali, Xiaoqing Liu, Michael Adamian, Shahrokh C. Khani, Xiaoyun Xu, Mei-Hong Tan, Michael A. Sandberg, Tiansen Li
Publikováno v:
Gene therapy
Aryl hydrocarbon receptor-interacting protein-like 1 (AIPL1) is required for the biosynthesis of photoreceptor phosphodiesterase (PDE). Gene defects in AIPL1 cause a heterogeneous set of conditions ranging from Leber's congenital amaurosis (LCA), the
Autor:
Szilard Kiss, Norman Michaud, Michael Adamian, Ivana K. Kim, Margaret M. DeAngelis, Tiansen Li, Edward Connolly, Hyeong Gon Yu, Evangelos S. Gragoudas, Xiaoqing Liu, O. V. Bulgakov, Joan W. Miller
Publikováno v:
Investigative ophthalmologyvisual science. 49(6)
PURPOSE. Age-related degradation of the elastic lamina in Bruch’s membrane may have a permissive effect on the growth of choroidal neovascularization (CNV). This study investigated the influence of defective elastic fiber maintenance in the develop
Autor:
O. V. Bulgakov, Joyce E. Young, Tiansen Li, Michael Adamian, Alexander J. Smith, Eileen M. Kasperek, Basil S. Pawlyk, Xun Sun, Shahrokh C. Khani, Robin R. Ali
Publikováno v:
Investigative ophthalmologyvisual science. 48(9)
PURPOSE. Gene therapy for retinal degeneration requires well-defined promoters that drive expression in rod and cone photoreceptors. This study was undertaken to develop short, active derivatives of the human rhodopsin kinase (RK) gene promoter for t
Autor:
V N, Pashkov, G P, Tsiuriupa, N B, Griko, O V, Bulgakov, N V, Rudenko, E B, Iakhnina, E V, Grishin
Publikováno v:
Bioorganicheskaia khimiia. 22(6)
Hybridoma lines producing anti-idiotypic monoclonal antibodies (AImAbs) were prepared by fusing splenocytes of mice immunized with alpha-latrotoxin (LT) and P3-X63Ag8.653 myeloma line cells. AImAbs (1) bind to the rat brain synaptosomes, (2) do not a