Wednesday, April 4, 2018

The transcriptional regulator CCCTC-binding factor limits oxidative stress in endothelial cells

Anna R Roy, Abdalla Ahmed, Peter V DiStefano, Lijun Chi, Nadiya Khyzha, Niels Galjart, Michael D Wilson, Jason E Fish and Paul Delgado Olguin; J. Biol. Chem., First Published on April 2, 2018 doi: 10.1074/jbc.M117.814699

Ctcf promotes vascular development, and limits oxidative stress in endothelial cells. These results reveal a function for Ctcf in vascular development, and suggest a potential mechanism for endothelial dysfunction in FRDA.

Idebenone: Novel Strategies to Improve Its Systemic and Local Efficacy

Lucia Montenegro, Rita Turnaturi, Carmela Parenti and Lorella Pasquinucci; Nanomaterials 2018, 8(2), 87; doi:10.3390/nano8020087

Due to IDE poor bioavailability after oral and topical administration, many researchers attempted different strategies to increase IDE efficacy in the treatment of neurodegenerative diseases and skin disorders. IDE chemical modifications mainly focused on increasing IDE activity at cellular or mitochondrial level while nanotechnology approaches aimed at designing novel formulations to improve IDE systemic and local efficacy. The encouraging results obtained loading IDE in different delivery systems such as liposomes, cyclodextrins and lipid-based nanoparticles could open new perspectives in the therapeutic outcomes of this strong antioxidant agent

Impact of Mobility Device Use on Quality of Life in Children With Friedreich Ataxia

Resham Ejaz, MD, Shiyi Chen, MSc, Charles J. Isaacs, BA, , Amanda Carnevale, MSc, Judith Wilson, RN(EC), MN, NP-Paediatrics, Kristen George, NP-Paediatrics, Martin B. Delatycki, MBBS, FRACP, PhD, Susan L. Perlman, MD, Katherine D. Mathews, MD, George R. Wilmot, MD, PhD, J. Chad Hoyle, MD, Sub H. Subramony, MD, Theresa Zesiewicz, MD, Jennifer M. Farmer, MS, , David R. Lynch, MD, PhD, , , Grace Yoon, MD, FRCPC, Journal of Child Neurology First Published April 2, 2018 doi:10.1177/0883073818764941

Mobility device use is associated with significant worsening of all domains of quality of life in children with Friedreich ataxia.