17 papers · ranked by Valyu relevance
Artur V. Cideciyan, Samuel G. Jacobson, Alejandro J. Roman, Alexander Sumaroka + 6 more
'Alexander Sumaroka' 'Vivian Wu' 'Jason Charng' 'Brianna Lisi' 'Malgorzata Swider' 'Gustavo D. Aguirre' 'William A. Beltran'] A common inherited retinal disease is caused by mutations in RHO expressed in rod photoreceptors that provide vision in dim ambient light. Approximately half of all RHO mutations result in a…
Myra B. McGuinness, Rogan G. Fraser, Rose Tan, Chi D. Luu + 1 more
'Robyn H. Guymer'] Purpose To quantify the association between dark adaptation parameters and other clinical measures of visual function among people with and without early and intermediate age-related macular degeneration (AMD). Methods In this cross-sectional study, participants underwent multimodal imaging and…
Bo Lei, Steven Barnes
Purpose The rodent retina does not exhibit a positive OFF-response in the electroretinogram (ERG), which makes it difficult to evaluate its OFF-pathway functions in vivo. We studied the rod-driven OFF pathway responses by using a dark-adapted 10-Hz flicker ERG procedure in mouse. Materials and Methods Conventional ERGs…
Xufeng Dai, Hua Zhang, Ying He, Yan Qi + 3 more
'Stephan C.F. Neuhauss'] Early studies on Rpe65 knockout mice reported that remaining visual function was attributable to cone function. However, this finding has been challenged more and more as time has passed. Electroretinograms (ERGs) showed that rd12 mice, a spontaneous animal model of RPE65 Leber’s congenital…
Cláudia Pereira, Rita M. Reis, José B. Gama, Ricardo Celestino + 3 more
'Dhanya K. Cheerambathur' 'Ana X. Carvalho' 'Reto Gassmann'] Title: Summary The kinetochore is a dynamic multi-protein assembly that forms on each sister chromatid and interacts with microtubules of the mitotic spindle to drive chromosome segregation. In animals, kinetochores without attached microtubules expand their…
Yanjie Wang, Steven Nusinowitz, Xian-Jie Yang
Retinitis pigmentosa (RP) is an inherited retinal disease in which the loss of rod photoreceptors precedes cone photoreceptor degeneration. The neurocytokine ciliary neurotrophic factor (CNTF) can provide potent neuroprotection for photoreceptors in various retinal degeneration models and has thus been tested in…
Federica Genovese, Johannes Reisert, Vladimir J. Kefalov
The past decades have seen tremendous progress in our understanding of the function of photoreceptors and olfactory sensory neurons, uncovering the mechanisms that determine their properties and, ultimately, our ability to see and smell. This progress has been driven to a large degree by the powerful combination of…
Shuai Li, Joe Mitchell, Deidrie J. Briggs, Jaime K. Young + 3 more
In this study we characterized the two primary rod spherule morphologies in the mouse retina and compared them to the retracted rod spherule in a disease model. The Mus musculus retina has an axonless rod morphology, and we report that synaptic structures are preserved in this population. This structure superficially…
Chanok Son, Hyo Kyung Lee, Hyoik Jang, Chul-Woo Park + 6 more
Objectives: Retinitis pigmentosa (RP) is commonly initiated by rod photoreceptor degeneration due to genetic mutations, followed by secondary cone loss and progressive blindness. Preserving rod function during the earlier stages of RP is a key therapeutic goal, as rod survival supports cone maintenance and delays…
János Lelkes, Bendegúz Dezső Bak, Tamás Kalmár-Nagy
Functionally graded materials have broad engineering applications including mechanical engineering, electronics, chemistry, and biomedical engineering. One notable advantage of such materials is that their stiffness distribution can be optimized to avoid stress concentration. A novel approach for solving the equations…
Amanda M Travis, Samiya Manocha, Jason R Willer, Timothy S Wessler + 4 more
'Nikolai P Skiba' 'Jillian N Pearring' 'Saikat Mukhopadhyay' 'Piali Sengupta'] The small GTPase Arl3 is important for the enrichment of lipidated proteins to primary cilia, including the outer segment of photoreceptors. Human mutations in the small GTPase Arl3 cause both autosomal recessive and dominant inherited…
Najate Aït-Ali, Thierry Léveillard
We studied the origin of rod-derived cone viability factor (RdCVF) during evolution. In mammals, the nucleoredoxin-like 1 gene (NXNL1) produces a truncated thioredoxin-like protein, RdCVF, by intron retention in rod photoreceptors of the retina. This protein prevents the secondary cone degeneration in animal models of…
Linjing Li, Kollu N. Rao, Hemant Khanna
X-linked retinitis pigmentosa 2 (XLRP2) patients and Rp2null mice exhibit severe cone photoreceptor degeneration. However, due to the paucity of cones in mammalian model systems, it is not clear how cones respond to the loss of RP2. Here we have used the Nrl-/- mice, which develop a rodless and short wavelength (S)…
William Ebo Annan, Emmanuel O. A. Asamani, Diana White, Ogugua Ndubuisi Okonkwo
'Ogugua Ndubuisi Okonkwo'] Retinal detachment (RD) is the separation of the neural layer from the retinal pigmented epithelium thereby preventing the supply of nutrients to the cells within the neural layer of the retina. In vertebrates, primary photoreceptor cells consisting of rods and cones undergo daily renewal of…
Ashley A. Rowe, Mauricio J. Velasquez, Jacob W. Aumeier, Sofia Reyes + 4 more
'Tiffany Yee' 'Emily R. Nettesheim' 'Jeffrey G. McDonald' 'Katherine J. Wert'] Neurodegenerative disorders such as Alzheimer’s disease and macular degeneration represent major sources of human suffering, yet factors influencing disease severity remain poorly understood. Sex has been implicated as one modifying factor.…
Meet K. Patel, Warlen Piedade, Jakub K. Famulski
Cone rod dystrophy (CRD) is a macular degeneration disorder characterized by initial cone cell photoreceptor degeneration and subsequently of rod photoreceptors. Mutations in CDHR1, a photoreceptor specific cadherin have been found to be associated with the incidence of cone-rod dystrophy and recapitulated in mouse…
Monica Aguilà, James Bellingham, Dimitra Athanasiou, Dalila Bevilacqua + 8 more
'Dalila Bevilacqua' 'Yanai Duran' 'Ryea Maswood' 'David A Parfitt' 'Takao Iwawaki' 'Giannis Spyrou' 'Alexander J Smith' 'Robin R Ali' 'Michael E Cheetham'] Title: Abstract Rhodopsin misfolding caused by the P23H mutation is a major cause of autosomal dominant retinitis pigmentosa (adRP). To date, there are no effective…