Inherited retinal diseases: therapeutics, clinical trials and end points—a review

M Georgiou, K Fu**ami… - Clinical & Experimental …, 2021 - Wiley Online Library
Inherited retinal diseases (IRDs) are a clinically and genetically heterogeneous group of
disorders characterised by photoreceptor degeneration or dysfunction. These disorders …

Progressive cone and cone-rod dystrophies: clinical features, molecular genetics and prospects for therapy

JS Gill, M Georgiou, A Kalitzeos, AT Moore… - British Journal of …, 2019 - bjo.bmj.com
Progressive cone and cone-rod dystrophies are a clinically and genetically heterogeneous
group of inherited retinal diseases characterised by cone photoreceptor degeneration …

Cell atlas of the human fovea and peripheral retina

W Yan, YR Peng, T van Zyl, A Regev, K Shekhar… - Scientific reports, 2020 - nature.com
Most irreversible blindness results from retinal disease. To advance our understanding of
the etiology of blinding diseases, we used single-cell RNA-sequencing (scRNA-seq) to …

Phenoty** and genoty** inherited retinal diseases: Molecular genetics, clinical and imaging features, and therapeutics of macular dystrophies, cone and cone-rod …

M Georgiou, AG Robson, K Fu**ami… - Progress in retinal and …, 2024 - Elsevier
Inherited retinal diseases (IRD) are a leading cause of blindness in the working age
population and children. The scope of this review is to familiarise clinicians and scientists …

Structure and function of ABCA4 and its role in the visual cycle and Stargardt macular degeneration

RS Molday, FA Garces, JF Scortecci… - Progress in retinal and eye …, 2022 - Elsevier
ABCA4 is a member of the superfamily of ATP-binding cassette (ABC) transporters that is
preferentially localized along the rim region of rod and cone photoreceptor outer segment …

Noninvasive imaging of the human rod photoreceptor mosaic using a confocal adaptive optics scanning ophthalmoscope

A Dubra, Y Sulai, JL Norris, RF Cooper… - Biomedical optics …, 2011 - opg.optica.org
The rod photoreceptors are implicated in a number of devastating retinal diseases.
However, routine imaging of these cells has remained elusive, even with the advent of …

Photoreceptor cilia and retinal ciliopathies

KM Bujakowska, Q Liu… - Cold Spring Harbor …, 2017 - cshperspectives.cshlp.org
Photoreceptors are sensory neurons designed to convert light stimuli into neurological
responses. This process, called phototransduction, takes place in the outer segments (OS) …

A review of the mechanisms of cone degeneration in retinitis pigmentosa

DS Narayan, JPM Wood, G Chidlow… - Acta …, 2016 - Wiley Online Library
Retinitis pigmentosa (RP) is an inherited condition that features degeneration of rod and
cone photoreceptors. In all forms of RP, the genetic mutation is expressed exclusively in …

Do you know the sex of your cells?

K Shah, CE McCormack… - American Journal of …, 2014 - journals.physiology.org
Do you know the sex of your cells? Not a question that is frequently heard around the lab
bench, yet thanks to recent research is probably one that should be asked. It is self-evident …

Gene therapy rescues cone function in congenital achromatopsia

AM Komáromy, JJ Alexander, JS Rowlan… - Human molecular …, 2010 - academic.oup.com
The successful restoration of visual function with recombinant adeno-associated virus
(rAAV)-mediated gene replacement therapy in animals and humans with an inherited …