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

Adaptive optimization of the OXPHOS assembly line partially compensates lrpprc-dependent mitochondrial translation defects in mice

OXPHOS 装配线的自适应优化部分补偿了小鼠中 lrpprc 依赖的线粒体翻译缺陷

Alexanne Cuillerier, Matthieu Ruiz, Caroline Daneault, Anik Forest, Jenna Rossi, Goutham Vasam, George Cairns, Virgilio Cadete; LSFC Consortium; Christine Des Rosiers, Yan Burelle

Lipidomics unveils lipid dyshomeostasis and low circulating plasmalogens as biomarkers in a monogenic mitochondrial disorder

脂质组学揭示脂质失衡和低循环缩醛磷脂是单基因线粒体疾病的生物标志物

Matthieu Ruiz, Alexanne Cuillerier, Caroline Daneault, Sonia Deschênes, Isabelle Robillard Frayne, Bertrand Bouchard, Anik Forest, Julie Thompson Legault; LSFC Consortium; Frederic M Vaz, John D Rioux, Yan Burelle, Christine Des Rosiers

mTORC1 is required for expression of LRPPRC and cytochrome- c oxidase but not HIF-1α in Leigh syndrome French Canadian type patient fibroblasts

mTORC1 是 Leigh 综合征法裔加拿大型患者成纤维细胞中 LRPPRC 和细胞色素 c 氧化酶表达所必需的,但不是 HIF-1α 表达所必需的

Yvette Mukaneza, Aaron Cohen, Marie-Ève Rivard, Jessica Tardif, Sonia Deschênes, Matthieu Ruiz; LSFC Consortium; Catherine Laprise, Christine Des Rosiers, Lise Coderre

Loss of hepatic LRPPRC alters mitochondrial bioenergetics, regulation of permeability transition and trans-membrane ROS diffusion

肝脏 LRPPRC 的缺失会改变线粒体生物能量学、通透性转换调节和跨膜 ROS 扩散

Alexanne Cuillerier, Shamisa Honarmand, Virgilio J J Cadete, Matthieu Ruiz, Anik Forest, Sonia Deschênes, Claudine Beauchamp; LSFC Consortium; Guy Charron, John D Rioux, Christine Des Rosiers, Eric A Shoubridge, Yan Burelle

LRPPRC and SLIRP interact in a ribonucleoprotein complex that regulates posttranscriptional gene expression in mitochondria

LRPPRC 和 SLIRP 在核糖核蛋白复合物中相互作用,调节线粒体中的转录后基因表达

Florin Sasarman, Catherine Brunel-Guitton, Hana Antonicka, Timothy Wai, Eric A Shoubridge; LSFC Consortium