Results 71 to 80 of about 21,042 (237)

Assessing the Nature of Human Brain‐Derived Extracellular Vesicles on Synaptic Activity Via the Development of an Air‐liquid Microfluidic Platform

Advanced Science, EarlyView.
This seminal study highlights the interwoven and tightly regulated functions of brain‐derived extracellular vesicles. Using microfluidics, proteomics, and AI‐based electric signal analyses of cortical brain explants, this work provides a useful resource dataset revealing the composition of human brain‐derived extracellular vesicles obtained from ...
Corentin Bernou, Yukiko Iwasaki, Willy Lutz, Glaucia Almeida, Emilie Béchard, François Delalande, Magali Rompais, Jules Bouget, Barbara Gorda, Chantal Cazevieille, Yonis Bare, Christine Carapito, Sophie Colomb, Gowrishankar Ganesh, Raphael Gaudin   +14 more
wiley   +1 more source

Intracellular Mechanical Stress‐Mediated Autophagy Cell Death via Nanospikes for Cancer Treatment

Advanced Science, EarlyView.
Nanospikes with tunable morphology at the nanoscale induce lysosomal membrane damage by generating localized mechanical stress, activating Galectin‐3 (Gal3)‐Trim16‐mediated autophagic cell death. Finite element analysis and laser‐triggered spike ablation enable precise control of intracellular force signaling and cytotoxicity, offering a ...
Yingze Li, Zihan Guo, Jiawei Fan, Ruimei Zhou, Jiayan Li, Zhixiang Hu, Weicheng Gu, Mengge Zheng, Chang Xu, Yichao Tang, Chang Chen, Yu Cheng   +11 more
wiley   +1 more source

Limitations of drug registries to evaluate orphan medicinal products for the treatment of lysosomal storage disorders [PDF]

, 2011
Carla E. M. Hollak, Johannes M. F. G. Aerts, Ségolène Aymé, Jeremy Manuel   +3 more
openalex   +1 more source

Purifying and profiling lysosomes to expand understanding of lysosomal dysfunction–associated diseases

The Journal of Clinical Investigation
Lysosome storage dysfunction plays a central role in numerous human diseases, but a lack of appropriate tools has hindered lysosomal content profiling in clinical settings. In this issue of the JCI, Saarela et al. introduce a method called tagless LysoIP
Ali Shilatifard, Issam Ben-Sahra
doaj   +1 more source

Impact of ER stress and the unfolded protein response on Fabry disease

EBioMedicine
Summary: Fabry disease (FD) is a lysosomal storage disorder caused by pathogenic missense and nonsense variants in the α-galactosidase A (GLA) gene, leading to absent or reduced enzyme activity.
Malte Lenders, Elisa Rudolph, Eva Brand
doaj   +1 more source

Mechanical Stress Triggers Premature Senescence in Cardiac Fibroblasts

Advanced Science, EarlyView.
Cellular senescence contributes to disease burden in cardiovascular disease (CVD) and aging, highlighting the need to understand its induction. In primary cardiac fibroblasts, reduced strain and increased frequency, mimicking CVD, elicit a distinct senescent phenotype compared to oxidative stress.
Stephanie E. Schneider, Adrienne K. Scott, Katie M. Gallagher, Emily Y. Miller, Soham Ghosh, Corey P. Neu   +5 more
wiley   +1 more source

Functional screening of lysosomal storage disorder genes identifies modifiers of alpha-synuclein neurotoxicity. [PDF]

PLoS Genet, 2023
Yu M, Ye H, De-Paula RB, Mangleburg CG, Wu T, Lee TV, Li Y, Duong D, Phillips B, Cruchaga C, Allen GI, Seyfried NT, Al-Ramahi I, Botas J, Shulman JM.   +14 more
europepmc   +1 more source

USP11‐PGAM5 Axis Promotes Neurotoxic Astrocyte Reactivity by Aggravating the mtDNA‐cGAS‐STING Pathway After Intracerebral Hemorrhage

Advanced Science, EarlyView.
This study reveals that the deubiquitinase USP11 stabilizes PGAM5, triggering neurotoxic astrocyte transformation after intracerebral hemorrhage. PGAM5 promotes mPTP opening and Drp1 dephosphorylation, synergistically amplifying mtDNA leakage into the cytosol and leading to cGAS–STING hyperactivation.
Jiaqing He, Zijuan Qin, Yaning Cai, Haixiao Liu, Tinghao Wang, Qing Hu, Yanni Xu, Pan Yang, Xun Wu, Yan Qu, Wei Guo   +10 more
wiley   +1 more source

Multiplexing Iduronate-2-Sulphatase (MPS-II) into a 7-Plex Lysosomal Storage Disorder MS/MS Assay Using Cold-Induced Phase Separation. [PDF]

Int J Neonatal Screen, 2023
Courtney E, Pickens CA, Cuthbert C, Petritis K.   +3 more
europepmc   +1 more source

Evaluation of the lysosome-associated membrane protein LAMP-2 as a marker for lysosomal storage disorders [PDF]

, 1998
Chi T. Hua, John J. Hopwood, Sven R. Carlsson, R. J. Harris, Peter J. Meikle   +4 more
openalex   +1 more source