Altered expression of beta-galactosidase-1-like protein 3 (Glb1l3) in the retinal pigment epithelium (RPE)-specific 65-kDa protein knockout mouse model of Leber's congenital amaurosis

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Publications associées (30)

Gene Editing Preserves Visual Functions in a Mouse Model of Retinal Degeneration

Naïg Aurelia Ludmilla Chenais, Diego Ghezzi, Paola Vagni, Tommaso Marchetti

Inherited retinal dystrophies (IRDs) are a large and heterogeneous group of degenerative diseases caused by mutations in various genes. Given the favorable anatomical and immunological characteristics of the eye, gene therapy holds great potential for thei ...

2019

Capacitive-like photovoltaic epiretinal stimulation enhances and narrows the network-mediated activity of retinal ganglion cells by recruiting the lateral inhibitory network

Naïg Aurelia Ludmilla Chenais, Marta Jole Ildelfonsa Airaghi Leccardi, Diego Ghezzi

Photovoltaic retinal prostheses theoretically offer the possibility of a standalone high-resolution electrical stimulation of the retina. However, achieving focused epiretinal stimulation is particularly challenging, because of axonal activation and electr ...

2019

The virtuous cycle of human genetics and mouse models in drug discovery

Johan Auwerx

Ongoing studies in many species seek to understand the origins, architecture and consequences of phenotypic variation under normal and dysfunctional conditions, with the aim of identifying targets for intervention that can prevent, stabilize or reverse dis ...

NATURE PUBLISHING GROUP2019

Glutaredoxin1 Diminishes Amyloid Beta-Mediated Oxidation of F-Actin and Reverses Cognitive Deficits in an Alzheimer's Disease Mouse Model

Bernard Schneider

Aims: Reactive oxygen species (ROS) generated during Alzheimer's disease (AD) pathogenesis through multiple sources are implicated in synaptic pathology observed in the disease. We have previously shown F-actin disassembly in dendritic spines in early AD ( ...

2019

Brain Dynamics: Global Pulse and Brain State Switching

Dimitri Nestor Alice Van De Ville

A major challenge in systems-level neuroscience is to understand the dynamic formation and succession of brain states. A new study has extracted reproducible brain states from mouse resting-state fMRI data, revealing interactions between occurrences of the ...

CELL PRESS2019

Supervised learning to quantify amyloidosis in whole brains of an Alzheimer's disease mouse model acquired with optical projection tomography

Theo Lasser, Dimitri Nestor Alice Van De Ville, Aleksandra Radenovic, Paul James Marchand, Virginie Sophie Uhlmann, David Nguyen, Arielle Louise Planchette

Alzheimer's disease (AD) is characterized by amyloidosis of brain tissues. This phenomenon is studied with genetically-modified mouse models. We propose a method to quantify amyloidosis in whole 5xFAD mouse brains, a model of AD. We use optical projection ...

2019

Impact of MCT1 Haploinsufficiency on the Mouse Retina

Sylvain Eric Lengacher, Pierre Magistretti, Luc Pellerin

The monocarboxylate transporter 1 (MCT1) is highly expressed in the outer retina, suggesting that it plays a critical role in photoreceptors. We examined MCT1+/- heterozygotes, which express half of the normal complement of MCT1. The MCT1(+/-) retina devel ...

SPRINGER INTERNATIONAL PUBLISHING AG2018

Disrupting Y-Box-Binding Protein 1 Function Using OSU-03012 Prevents Endometriosis Progression in In Vitro and In Vivo Models

The objective of the present study was to test the ability of OSU-03012 (2-amino-N-[4-[5-phenanthren-2-yl-3-(trifluoromethyl)pyrazol-1-yl]phenyl]acetamide), a novel and potent celecoxib-derivative, to impair endometriosis progression in in vitro and in viv ...

Sage Publications Inc2017

From retinotopic processing to nonretinotopic representation

Marc Michael Lauffs

Encoding of visual information in the brain is retinotopic: Neighboring points in the visual field are mapped onto neighboring photoreceptors in the retina, and these neighborhood relations are maintained in the early stages of cortical processing. However ...

EPFL2017

Social isolation stress and chronic glutathione deficiency have a common effect on the glutamine-to-glutamate ratio and myo-inositol concentration in the mouse frontal cortex

Rolf Gruetter, João Miguel das Neves Duarte, Alberto Corcoba, Kim Do

Environmental stress can interact with genetic predisposition to increase the risk of developing psychopathology. In this work, we tested the hypothesis that social isolation stress interacts with impaired glutathione synthesis and have cumulative effects ...

Wiley-Blackwell2017

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