Results 181 to 190 of about 483,484 (261)

The First Heterozygous <i>TWNK</i> Nonsense Mutation Associated with Progressive External Ophthalmoplegia: Evidence for a New Piece in the Puzzle of Mitochondrial Diseases. [PDF]

Biomolecules
Lopergolo D, Berti G, Gallus GN, Bianchi S, Santorelli FM, Malandrini A, De Stefano N.   +6 more
europepmc   +1 more source

Agnuside Stabilizes the Complex I Assembly Factor NDUFAF6 to Reinforce Mitochondrial Efficiency and Thermogenic Responsiveness

Advanced Science, EarlyView.
ABSTRACT Brown and beige adipocytes dissipate energy as heat, yet effective strategies to enhance their mitochondrial efficiency remain limited. Here, we identify Agnuside (AGN) as a selective stabilizer of the complex I assembly factor NDUFAF6. AGN directly binds cytosolic NDUFAF6, suppresses its ubiquitination, prolongs its half‐life, and facilitates
Qingwen Zhao, Li Xie, Qianzhuo Wang, Shuying Dai, Bei Li, Yingjuan Zhang, Zhe Sun, Yue Gao   +7 more
wiley   +1 more source

Apoptosis-inducing factor (AIF) at the crossroad of cell survival and cell death: implications for cancer and mitochondrial diseases. [PDF]

Cell Commun Signal
Nguyen TNA, Lai HT, Fernandes R, Dall'Olio FG, Blériot C, Ha-Duong T, Brenner C.   +6 more
europepmc   +1 more source

Phosphorylation‐Facilitated CKB Lactylation At K11 By GCN5 Enhances Creatine Kinase Activity and Mitigates Neuronal Damage After Cerebral Ischemia‐Reperfusion

Advanced Science, EarlyView.
This study uncovers hierarchical coordination between K11 lactylation and S199 phosphorylation of CKB in cerebral ischemia‐reperfusion injury. Such dual modifications potentiate CKB enzymatic function, remodel energy metabolism, alleviate oxidative stress and neuronal damage, and represent a viable therapeutic target for stroke treatment.
Chao Duan, Ruolin Zhang, Shihui Ding, Ying Feng, Tao Shen, Ziwei Wang, Wendai Bao, Ke Shui, Jun Qin, Jun Chen, Min Zhang, Zijing Ren, Dongmei Zhao, Xingrong Guo, Zhibing Ai, Xin Yang, Peiyang Zhou, Zhiqiang Dong   +17 more
wiley   +1 more source

Polydatin and Nicotinamide Prevent Iron Accumulation and Lipid Peroxidation in Cellular Models of Mitochondrial Diseases. [PDF]

Antioxidants (Basel)
Cilleros-Holgado P, Gómez-Fernández D, Piñero-Pérez R, Romero-Domínguez JM, Reche-López D, Álvarez-Córdoba M, Romero-González A, López-Cabrera A, De Oliveira MC, Rodríguez-Sacristán A, González-Granero S, García-Verdugo JM, Sánchez-Alcázar JA.   +12 more
europepmc   +1 more source

A 3D Human Bone and Bone Marrow‐on‐a‐Chip Model for In Vitro Bone Remodeling and Immune Cell Maintenance

Advanced Science, EarlyView.
This study presents a human Bone (and Bone Marrow)‐on‐a‐Chip model based on native human bone scaffolds and autologous cells. Dynamic perfusion and sequential cell seeding replicate the physiological bone remodeling process in vitro, enabling the long‐term culture of functional, mature bone marrow immune subpopulations.
Nina Stelzer, Melanie‐Jasmin Ort, Kristian Händler, Emely Bortel, Ioanna Maria Dimitriou, Martin Textor, Georg N. Duda, Janosch Schoon, Uwe Marx, Uwe Kornak, Annika Winter, Bernhard Hesse, Stefanie Donner, Sebastian Hardt, Oliver Klein, Simon Reinke, Malte Spielmann, Sven Geißler   +17 more
wiley   +1 more source

Clinical drivers of hospitalisation in patients with mitochondrial diseases. [PDF]

BMJ Neurol Open
Haque S, Crawley K, Davis R, Schofield D, Shrestha R, Sue CM.   +5 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

Endocrine Disorders in Children with Primary Mitochondrial Diseases: Single Center Experience [PDF]

J Clin Res Pediatr Endocrinol
Papatya Çakır ED, Ersoy M, Çakır Biçer N, Gedikbaşı A.   +3 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