[HTML][HTML] Feedback regulation of RTK signaling in development

CL Neben, M Lo, N Jura, OD Klein - Developmental biology, 2019 - Elsevier
Precise regulation of the amplitude and duration of receptor tyrosine kinase (RTK) signaling
is critical for the execution of cellular programs and behaviors. Understanding these control …

Endoplasmic reticulum stress: New insights into the pathogenesis and treatment of retinal degenerative diseases

MS Gorbatyuk, CR Starr, OS Gorbatyuk - Progress in retinal and eye …, 2020 - Elsevier
Physiological equilibrium in the retina depends on coordinated work between rod and cone
photoreceptors and can be compromised by the expression of mutant proteins leading to …

Gene Therapy Using a miniCEP290 Fragment Delays Photoreceptor Degeneration in a Mouse Model of Leber Congenital Amaurosis

W Zhang, L Li, Q Su, G Gao, H Khanna - Human gene therapy, 2018 - liebertpub.com
Mutations in the cilia-centrosomal protein CEP290 are frequently observed in autosomal
recessive childhood blindness disorder Leber congenital amaurosis (LCA). No treatment or …

A CEP290 C-terminal domain complements the mutant CEP290 of Rd16 mice in trans and rescues retinal degeneration

S Mookherjee, HY Chen, K Isgrig, W Yu, S Hiriyanna… - Cell reports, 2018 - cell.com
Mutations in CEP290 cause ciliogenesis defects, leading to diverse clinical phenotypes,
including Leber congenital amaurosis (LCA). Gene therapy for CEP290-associated …

GRK2 constitutively governs peripheral delta opioid receptor activity

AD Brackley, R Gomez, AN Akopian, MA Henry… - Cell reports, 2016 - cell.com
Opioids remain the standard for analgesic care; however, adverse effects of systemic
treatments contraindicate long-term administration. While most clinical opioids target mu …

Ablation of retinal ciliopathy protein RPGR results in altered photoreceptor ciliary composition

KN Rao, L Li, M Anand, H Khanna - Scientific reports, 2015 - nature.com
Cilia regulate several developmental and homeostatic pathways that are critical to survival.
Sensory cilia of photoreceptors regulate phototransduction cascade for visual processing …

Translational attenuation and retinal degeneration in mice with an active integrated stress response

CR Starr, PM Pitale, M Gorbatyuk - Cell death & disease, 2018 - nature.com
An integrated stress response (ISR), identified in several different animal models of inherited
retinal degeneration (IRD), is activated following various cellular stresses. The ISR results in …

Loss of human disease protein retinitis pigmentosa GTPase regulator (RPGR) differentially affects rod or cone-enriched retina

KN Rao, L Li, W Zhang, RS Brush… - Human Molecular …, 2016 - academic.oup.com
It is unclear how genes, such as RPGR (retinitis pigmentosa guanine triphosphatase
regulator) that are expressed in both rods and cones, cause variable disease pathogenesis …

Delineating the role of eIF2α in retinal degeneration

CR Starr, MS Gorbatyuk - Cell Death & Disease, 2019 - nature.com
Activation of the unfolded protein response has been detected in various animal models of
retinal degeneration. The PERK branch converges on eIF2α to regulate protein synthesis …

[HTML][HTML] Role of translational attenuation in inherited retinal degeneration

CR Starr, CNA Nyankerh, X Qi, Y Hu… - … & Visual Science, 2019 - tvst.arvojournals.org
Purpose: We reported previously that retinas of mice with inherited retinal degeneration
make less protein than retinas of normal mice. Despite recent studies suggesting that …