Results 1 to 10 of about 2,946,385 (264)

Targeting mitochondrial deubiquitinase USP30 to induce mitophagy in heteroplasmic mitochondrial diseases. [PDF]

Pharmacol Rep
Pinho BR, Martins V, Chacko AR, Nogueira C, Duchen MR, Oliveira JMA.   +5 more
europepmc   +1 more source

Copper‐Driven Epithelial Barrier Disruption: A Novel Mechanism of COPD Acute Exacerbations Mediated by the TNF‐α/ATP7A Axis

Advanced Science, EarlyView.
This study reveals a copper‐driven mechanism of COPD acute exacerbations mediated by the TNF‐α/ATP7A axis. TNF‐α suppresses the copper transporter ATP7A via competitive NF‐κB/CREB1/CBP signaling, resulting in intracellular copper accumulation, mitochondrial oxidative stress, and epithelial barrier disruption.
Xinru Xiao, Yongzhe Hao, Ziqi Ding, Zhipeng Wang, Yujia Shi, Yanhua Huang, Chunjian Qi, Qian Zhang   +7 more
wiley   +1 more source

Nutrient and endocrine factors affecting impaired growth in pediatric mitochondrial diseases. [PDF]

Endocrinol Diabetes Metab Case Rep
Yagasaki H, Narusawa H, Watanabe D, Sano F, Fujioka K, Mizorogi S, Kaga Y, Inukai T.   +7 more
europepmc   +1 more source

Mr BMT Achieves Systemic Macrophage Replacement With Preservation of Tissue Homeostasis

Advanced Science, EarlyView.
Microglia replacement by bone marrow transplantation (Mr BMT) enables systemic replacement of tissue‐resident macrophages. Despite persistent macrophage and tissue remodeling across multiple organs, core biological functions and innate immune responses remain preserved, supporting long‐term maintenance of organismal homeostasis and the therapeutic ...
Yufei Xu, Miaozhan Zou, Yunshang Bai, Yang He, Zhihao Jin, Baozhi Yang, Jinjin Du, Pei Ouyang, Yuanyuan Cai, Yuheng Zhang, Bingying Du, Chengcheng Ge, Jian‐an Gong, Xiaoyu Li, Yuanyi Zheng, Jing Ding, Bo Peng, Yanxia Rao   +17 more
wiley   +1 more source

Mitochondrial Diseases: Molecular Pathogenesis and Therapeutic Advances. [PDF]

MedComm (2020)
Mei J, Ding P, Gao C, Zhou J, Li Z, Zhang C, Gao J.   +6 more
europepmc   +1 more source

Mitochondria and Neuromast Tagging With Fluorescent Gallium‐Triapine Analogues: In Cellulo MP FLIM and Zebrafish Live Imaging

Advanced Science, EarlyView.
Fluorescent BODIPY‐conjugated thiosemicarbazone ligands and their Ga(III), In(III), and Fe(III) complexes, inspired by Triapine, are developed as theranostic agents. Multiphoton FLIM and confocal microscopy in cancer cells and zebrafish reveal real‐time uptake, mitochondrial localisation, and whilst spectroscopic assays indicated preserved complex ...
Megan J. Green, Michael W. Jones, Merissa Saleem, Haobo Ge, Melita Tvardauskaite, Julia Dudzic, Fernando Cortezon‐Tamarit, Rory L. Arrowsmith, Charareh Pourzand, Gabriele Kociock‐Kohn, Nicholas H. Rees, Jonathan R. Dilworth, Stephen Faulkner, Stanley W. Botchway, David Gurevich, Sofia I. Pascu   +15 more
wiley   +1 more source

Evaluating a Coenzyme Q10-Based Food for Special Medical Purpose, for Mitochondrial Diseases Management: An Open-Label, Pilot Trial. [PDF]

Int J Mol Sci
Chico L, Lopriore P, Cecchi G, Meli A, Bernardini C, Balestrini L, Polzella M, Montano V, Mancuso M.   +8 more
europepmc   +1 more source

NeuroSuite for Long‐Term Functional and Structural Studies of Air‐Liquid Interface Cerebral Organoids

Advanced Science, EarlyView.
NeuroSuite provides a modular hardware‐software platform integrating Neuroweb and NeuroMaps to enable long‐term, in situ electrophysiological interrogation of air–liquid interface organoid slices while preserving tissue architecture. Its components can be used together or independently to capture real‐time activity, spatial network dynamics, and ...
Belquis Haider, Sagnik Middya, Daniel J. Lloyd‐Davies‐Sánchez, Nino F. Läubli, Sulay Vora, Jakob Träuble, Rohan N. Krajeski, Yuqing Feng, Daniel Duncko, Josiah S. Riley, Rubén Ruiz‐Mateos Serrano, Ole Paulsen, Madeline A. Lancaster, George G. Malliaras, Gabriele S. Kaminski Schierle   +14 more
wiley   +1 more source

ATAD3 duplications bridge mitochondrial diseases and Aicardi-Goutières syndrome. [PDF]

Dev Med Child Neurol
Planté-Bordeneuve P, Bérat CM, Hanein S, Gitiaux C, ATAD3 Study Group, Steffann J, Desguerre I, Rötig A, Boddaert N, Barcia G.   +9 more
europepmc   +1 more source

Combating Inflammation and Promoting Anabolism in Osteoarthritic Cartilage Defect With an MMP13‐Sensing Dual‐Drug Scaffold

Advanced Science, EarlyView.
ABSTRACT Osteoarthritis (OA) is a widespread degenerative joint condition marked by progressive cartilage breakdown, and a chronic inflammatory microenvironment, where conventional therapies largely fail to halt disease progression. To address this unmet need, an intra‐articularly implantable, disease‐responsive scaffold was developed for combinatorial
Zhen Zhang, Bangheng Liu, Yulei Mu, Huiqun Zhou, Liang Ma, Chenjie Xu, Dong‐An Wang   +6 more
wiley   +1 more source