Results 191 to 200 of about 156,351 (339)

Plasma membrane remodeling in GM2 gangliosidoses drives synaptic dysfunction.

PLoS Biology
Glycosphingolipids (GSL) are important bioactive membrane components. GSLs containing sialic acids, known as gangliosides, are highly abundant in the brain and diseases of ganglioside metabolism cause severe early-onset neurodegeneration. The ganglioside
Alex S Nicholson, David A Priestman, Robin Antrobus, James C Williamson, Reuben Bush, Shannon J McKie, Henry G Barrow, Emily Smith, Kostantin Dobrenis, Nicholas A Bright, Frances M Platt, Janet E Deane   +11 more
doaj   +1 more source

AXL Promotes Ischemic Myelin Repair Through Alleviating Myelin Debris Deposition and Lipid Droplets Accumulation

Advanced Science, EarlyView.
Microglial AXL drives white matter repair after stroke by orchestrating the cleanup of myelin debris. Mechanistically, AXL signals through EGR1 to boost Smpd1 transcription, regulating sphingolipid metabolism and preventing lipid droplet toxicity. Restoring the pathway with ASM therapy mitigates damage, positioning AXL as a key node for therapeutic ...
Junqiu Jia, Yonghui Gan, Jia Li, Lilin Li, Hailan Meng, Min Sun, Lei Ye, Rui Mao, Xiang Cao, Shengnan Xia, Xinyu Bao, Renyuan Liu, Meijuan Zhang, Yun Xu   +13 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

Case Report: Pancytopenia as an indicator for lysosomal storage disease (Gaucher's Disease) [PDF]

, 2019
Alberto Ortega-Rosales, Carlos Burneo-Rosales, Gilda Romero-Ulloa, Gabriela Burneo-Rosales   +3 more
openalex   +1 more source

A DNA Tetrahedron Delivery Asiatic Acid to Reprogram Mitochondrial Metabolism for Promoting Bone Regeneration via STAT3 Phosphorylation

Advanced Science, EarlyView.
A hyaluronic acid methacrylate (HAMA) hydrogel incorporating DNA tetrahedrons loaded with Asiatic acid (TDN@AA) was developed. HM‐TDN@AA promotes angiogenesis of endothelial cells (ECs), inhibits osteoclastogenesis from bone marrow–derived macrophages (BMDMs), and enhances osteogenesis of mesenchymal stem cells (MSCs) via STAT3‐mediated mitochondrial ...
Yiwen Huang, Yiming Zhang, Yisheng Feng, Qixiang Yang, Beiyuan Gao, Haiyang Xu, Cheng Huang, Kaili Lin, Yuanzhi Xu, Peiqi Zhu   +9 more
wiley   +1 more source

Methyl-β-cyclodextrin restores impaired autophagy flux in Niemann-Pick C1-deficient cells through activation of AMPK [PDF]

, 2017
Austin, Christopher P., Dai, Sheng, Dulcey, Andres E., Hu, Xin, Marugan, Juan, Ory, Daniel S., Porter, Forbes D., Wassif, Christopher A., Zheng, Wei   +8 more
core   +2 more sources

Trojan Horse Strategy: How Biomimetic Nanomedicine Remodels the Tumor Microenvironment

Advanced Science, EarlyView.
This review focuses on biomimetic nanomedicines for tumor microenvironment (TME) remodeling, covering their diverse biomimetic types, design principles, and mechanisms of immune cell reprogramming and reversal of immunosuppressive microenvironments, with particular emphasis on their application in synergistic immunotherapy.
Wanrong Wang, Siqi Mu, Juan Zhang, Guisong Shan, Xueqian Li, Ran Wang, Tao Guo, Xiaoyan He   +7 more
wiley   +1 more source

Novel Fused Pyrazole Amide Analogs as Glucosylceramide Synthase Inhibitors for Treating Lysosomal Storage Diseases, Neurodegenerative Diseases, Cystic Diseases, and Cancer. [PDF]

ACS Med Chem Lett, 2023
Sabnis RW.
europepmc   +1 more source

Mutations in the genes of lysosomal storage diseases as a risk factor for the development of Parkinson’s disease [PDF]

Anton Emelyanov, M. V. Beletskaya, Konstantin Senkevich, A. Lavrinova, Anton Tyurin, Irina Miliukhina, Alla Timofeeva, A. V. Amelin, С. Н. Пчелина   +8 more
openalex   +1 more source

Precision‐Engineered Silver Single‐Atom Carbon Dot Nanozymes for Theranostic Management of Acute Kidney Injury

Advanced Science, EarlyView.
Mitochondria‐targeted silver single‐atom carbon dot (T‐AgSA‐CDs) nanozymes enable dual SOD/GPx‐like ROS scavenging, real‐time imaging, and therapeutic effects for acute kidney injury (AKI). Atomically dispersed Ag sites synergize with carbon dots to restore renal redox hemostasis, protect mitochondrial function, mitigate inflammation, facilitate post ...
Tianle Tang, Jun Zhang, Yan Wang, Guoping Chen, Kehui Yuan, Yuan He, Chenchen Li, Sumaira Hanif, Yang Yang, Yanli Wang, Pir Muhammad   +10 more
wiley   +1 more source