Results 101 to 110 of about 37,618 (246)

Lysosomes and Storage diseases

, 1970
info:eu-repo/semantics ...
Grégoire, Anne, Tondeur, Michel, Pohl, Sylvie, Dustin, Pierre   +3 more
openaire   +1 more source

ANGPTL4 Exacerbates Renal Injury in Diabetic Kidney Disease by Impairing Podocyte Lipophagy via Compromised Lysosomal Degradative Function

Advanced Science, EarlyView.
In diabetic kidney disease, elevated podocyte ANGPTL4 is linked to reduced TFEB nuclear localization and compromised lysosomal degradative function. These changes impair podocyte lipophagy and promote lipid‐droplet accumulation and podocyte injury, which may contribute to renal injury progression.
Xiaojing Liu, Shimin Jiang, Zhenkun Yang, Shunlai Shang, Jiayi Li, Guming Zou, Cheng Zhou, Wenge Li   +7 more
wiley   +1 more source

A Blood‐Derived Factor Rescues ALS: Platelet Factor 4 Activates OPTN‐Dependent Autophagy to Clear SOD1 Aggregates Independently of PINK1

Advanced Science, EarlyView.
Systemic platelet factor 4 (PF4) is significantly depleted in amyotrophic lateral sclerosis (ALS). Peripheral PF4 replenishment restores central proteostasis by driving OPTN‐dependent, PINK1‐independent selective autophagy in motor neurons. This intervention effectively clears toxic SOD1 aggregates, blunts glial activation, and preserves neuromuscular ...
Qingjian Xie, Yanbo Zhu, Wenhua Jiang, Haobo Xie, Yaojia Li, Mengjie Hu, Kezheng Li, Huan Yu, Yixuan Pan, Chaoyi Jiang, Xueqin Song, Dongsheng Fan, Binbin Deng   +12 more
wiley   +1 more source

Gene-modified neural progenitor cells for the treatment of neuropathic lysosomal storage diseases. [PDF]

Neural Regen Res, 2023
Mandolfo O, Bigger BW.
europepmc   +1 more source

An Aggregation‐Induced Polymerization Poly(Disulfide)‐Drug Nanoplatform for Autoimmune Uveitis Therapy via Inhibiting the cGAS‐STING Pathway

Advanced Science, EarlyView.
A cationic poly(disulfide)‐drug nanoplatform (LA/DexP) was developed to treat experimental autoimmune uveitis (EAU). With potent blood‐retinal barrier penetrability, LA/DexP releases DSP in response to high ROS and scavenges cfDNA to inhibit the cGAS‐STING signaling pathway.
Yuelan Wu, Wenbo Geng, Qinjin Dai, Wanyun Zhang, Yuxian Lai, Pei Zhang, Chunjiang Zhou, Yinuo Wang, Qingfeng Cao, Xiang Luo, Yujie Lai, Changwei Huang, Peizeng Yang   +12 more
wiley   +1 more source

The expanding boundaries of sphingolipid lysosomal storage diseases; insights from Niemann-Pick disease type C. [PDF]

Biochem Soc Trans, 2023
Platt FM.
europepmc   +1 more source

New Perspectives in Dried Blood Spot Biomarkers for Lysosomal Storage Diseases. [PDF]

Int J Mol Sci, 2023
Spiewak J, Doykov I, Papandreou A, Hällqvist J, Mills P, Clayton PT, Gissen P, Mills K, Heywood WE.   +8 more
europepmc   +1 more source

Biophysical Characterization and Proteomic Analysis of Small Extracellular Vesicles Derived From Different Neural Cell Lines

Advanced NanoBiomed Research, EarlyView.
This study systematically compares small extracellular vesicles (sEVs) derived from four neural cell lines, revealing how cellular origin shapes vesicle biophysical properties and proteomic cargo. Distinct, lineage‐specific signatures linked to neuronal, astrocytic, and microglial functions are identified, highlighting the importance of cell source ...
Muhammad Waqas Salim, Wei Zhang, Lyndsey E. Collins‐Praino, Andrew Care, Yuling Wang   +4 more
wiley   +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

COVID-19 and Vaccination Status in Lysosomal Storage Diseases: A Single-Center Experience. [PDF]

Turk Arch Pediatr, 2023
Yoldaş Çelik M, Canda E, Yazıcı H, Erdem F, Yazıcı Özkaya P, Bal Sahbudak Z, Kalkan Uçar S, Çoker M.   +7 more
europepmc   +1 more source