日期:
2020 年 — 2026 年
2020
2021
2022
2023
2024
2025
2026
影响因子:

The Wolfram-like variant WFS1E864K destabilizes MAM and compromises autophagy and mitophagy in human and mice

Wolfram 样变体 WFS1E864K 会破坏 MAM 的稳定性,并损害人和小鼠的自噬和线粒体自噬。

Simone Patergnani ,Méghane S Bataillard ,Alberto Danese ,Stacy Alves ,Chantal Cazevieille ,René Valéro ,Lisbeth Tranebjærg ,Tangui Maurice ,Paolo Pinton ,Benjamin Delprat ,Elodie M Richard

Wfs1E864K knock-in mice illuminate the fundamental role of Wfs1 in endocochlear potential production

Wfs1E864K敲入小鼠揭示了Wfs1在内耳电位产生中的基础作用。

Elodie M Richard # ,Emilie Brun # ,Julia Korchagina ,Lucie Crouzier ,Corentin Affortit ,Stacy Alves ,Chantal Cazevieille ,Anne-Laure Mausset-Bonnefont ,Marc Lenoir ,Jean-Luc Puel ,Tangui Maurice ,Marc Thiry ,Jing Wang ,Benjamin Delprat

Activation of the sigma-1 receptor chaperone alleviates symptoms of Wolfram syndrome in preclinical models

sigma-1 受体伴侣的激活可缓解临床前模型中的 Wolfram 综合征症状

Lucie Crouzier, Alberto Danese, Yuko Yasui, Elodie M Richard, Jean-Charles Liévens, Simone Patergnani, Simon Couly, Camille Diez, Morgane Denus, Nicolas Cubedo, Mireille Rossel, Marc Thiry, Tsung-Ping Su, Paolo Pinton, Tangui Maurice, Benjamin Delprat

Sigma-1 receptor agonist PRE-084 confers protection against TAR DNA-binding protein-43 toxicity through NRF2 signalling

Sigma-1 受体激动剂 PRE-084 通过 NRF2 信号传导保护机体免受 TAR DNA 结合蛋白 43 毒性的影响

Christelle Lasbleiz, Amandine Peyrel, Pauline Tarot, Jérôme Sarniguet, Lucie Crouzier, Nicolas Cubedo, Benjamin Delprat, Mireille Rossel, Tangui Maurice, Jean-Charles Liévens

Morphological, behavioral and cellular analyses revealed different phenotypes in Wolfram syndrome wfs1a and wfs1b zebrafish mutant lines

形态学、行为学和细胞分析揭示了 Wolfram 综合征 wfs1a 和 wfs1b 斑马鱼突变系的不同表型

Lucie Crouzier, Elodie M Richard, Camille Diez, Hala Alzaeem, Morgane Denus, Nicolas Cubedo, Thomas Delaunay, Emily Glendenning, Sarah Baxendale, Jean-Charles Liévens, Tanya T Whitfield, Tangui Maurice, Benjamin Delprat

NCS1 overexpression restored mitochondrial activity and behavioral alterations in a zebrafish model of Wolfram syndrome

NCS1 过表达恢复了 Wolfram 综合征斑马鱼模型中的线粒体活性和行为改变

Lucie Crouzier, Elodie M Richard, Camille Diez, Morgane Denus, Amandine Peyrel, Hala Alzaeem, Nicolas Cubedo, Thomas Delaunay, Tangui Maurice, Benjamin Delprat

Loss of Pde6a Induces Rod Outer Segment Shrinkage and Visual Alterations in pde6aQ70X Mutant Zebrafish, a Relevant Model of Retinal Dystrophy

Pde6a 缺失导致 pde6aQ70X 突变斑马鱼(视网膜营养不良的相关模型)视杆外节收缩和视觉改变

Lucie Crouzier, Camille Diez, Elodie M Richard, Nicolas Cubedo, Clément Barbereau, Mireille Rossel, Thomas Delaunay, Tangui Maurice, Benjamin Delprat

Sigma-1 Receptor Is Critical for Mitochondrial Activity and Unfolded Protein Response in Larval Zebrafish

Sigma-1 受体对斑马鱼幼虫的线粒体活性和未折叠蛋白反应至关重要

Lucie Crouzier, Morgane Denus, Elodie M Richard, Amarande Tavernier, Camille Diez, Nicolas Cubedo, Tangui Maurice, Benjamin Delprat

Sigma-1 (σ1) receptor activity is necessary for physiological brain plasticity in mice

Sigma-1(σ1)受体活性是小鼠生理大脑可塑性的必要条件

Lucie Crouzier, Simon Couly, Chloé Roques, Coralie Peter, Rislen Belkhiter, Maëva Arguel Jacquemin, Anna Bonetto, Benjamin Delprat, Tangui Maurice