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

BOLA3 and NFU1 link mitoribosome iron-sulfur cluster assembly to multiple mitochondrial dysfunctions syndrome

BOLA3 和 NFU1 将线粒体铁硫簇组装与多发性线粒体功能障碍综合征联系起来

Hui Zhong, Alexandre Janer, Oleh Khalimonchuk, Hana Antonicka, Eric A Shoubridge, Antoni Barrientos

The role of the mitochondrial outer membrane protein SLC25A46 in mitochondrial fission and fusion

线粒体外膜蛋白SLC25A46在线粒体裂变和融合中的作用

Jana Schuettpelz, Alexandre Janer, Hana Antonicka, Eric A Shoubridge

ESYT1 tethers the ER to mitochondria and is required for mitochondrial lipid and calcium homeostasis

ESYT1 将内质网与线粒体连接起来,是线粒体脂质和钙稳态所必需的

Alexandre Janer, Jordan L Morris, Michiel Krols, Hana Antonicka, Mari J Aaltonen, Zhen-Yuan Lin, Hanish Anand, Anne-Claude Gingras, Julien Prudent, Eric A Shoubridge

Loss of mitochondrial Chchd10 or Chchd2 in zebrafish leads to an ALS-like phenotype and Complex I deficiency independent of the mitochondrial integrated stress response

斑马鱼中线粒体 Chchd10 或 Chchd2 的缺失会导致 ALS 样表型和复合物 I 缺乏,与线粒体综合应激反应无关

Virginie Petel Légaré, Christian J Rampal, Tyler J N Gurberg, Mari J Aaltonen, Alexandre Janer, Lorne Zinman, Eric A Shoubridge, Gary A B Armstrong

A High-Density Human Mitochondrial Proximity Interaction Network

高密度人类线粒体邻近相互作用网络

Hana Antonicka, Zhen-Yuan Lin, Alexandre Janer, Mari J Aaltonen, Woranontee Weraarpachai, Anne-Claude Gingras, Eric A Shoubridge

Loss of CHCHD10-CHCHD2 complexes required for respiration underlies the pathogenicity of a CHCHD10 mutation in ALS

呼吸所需的 CHCHD10-CHCHD2 复合物的缺失是 ALS 中 CHCHD10 突变致病性的基础

Isabella R Straub, Alexandre Janer, Woranontee Weraarpachai, Lorne Zinman, Janice Robertson, Ekaterina Rogaeva, Eric A Shoubridge

SLC25A46 is required for mitochondrial lipid homeostasis and cristae maintenance and is responsible for Leigh syndrome

SLC25A46 是线粒体脂质稳态和嵴维持所必需的,并且是 Leigh 综合征的病因

Alexandre Janer, Julien Prudent, Vincent Paupe, Somayyeh Fahiminiya, Jacek Majewski, Nicolas Sgarioto, Christine Des Rosiers, Anik Forest, Zhen-Yuan Lin, Anne-Claude Gingras, Grant Mitchell, Heidi M McBride, Eric A Shoubridge

Interferon β induces clearance of mutant ataxin 7 and improves locomotion in SCA7 knock-in mice

干扰素 β 诱导清除突变型共济失调蛋白 7 并改善 SCA7 基因敲入小鼠的运动能力

Alice Chort, Sandro Alves, Martina Marinello, Béatrice Dufresnois, Jean-Gabriel Dornbierer, Christelle Tesson, Morwena Latouche, Darren P Baker, Martine Barkats, Khalid H El Hachimi, Merle Ruberg, Alexandre Janer, Giovanni Stevanin, Alexis Brice, Annie Sittler

SUMOylation attenuates the aggregation propensity and cellular toxicity of the polyglutamine expanded ataxin-7

SUMO 化可减弱多聚谷氨酰胺扩增的 Ataxin-7 的聚集倾向和细胞毒性

Alexandre Janer, Andreas Werner, Junko Takahashi-Fujigasaki, Aurélie Daret, Hiroto Fujigasaki, Koji Takada, Charles Duyckaerts, Alexis Brice, Anne Dejean, Annie Sittler