Results 91 to 100 of about 41,053 (230)

Galactosialidosis in two siblings

The Turkish Journal of Pediatrics, 1996
Galactosialidosis is a rare lysosomal storage disease associated with deficiencies of alpha-galactosidase and beta-neurominidase. In this report, two siblings with galactosialidosis, resembling Niemann-Pick disease with the presence of foamy cells
A Yuce, N Kocak, G T Besley
doaj  

Lipid Droplet‐Localized Spindle Apparatus Coiled‐Coil Protein 1 Regulates Lipid Droplet Distribution

Advanced Science, EarlyView.
SPDL1‐L, a long isoform of SPDL1, is identified as a bona fide lipid droplet‐associated protein that acts as a dynein adaptor to drive perinuclear clustering of lipid droplets. By linking lipid droplets to the microtubule network, SPDL1‐L extends the function of SPDL1 beyond mitosis and contributes to lipid droplet organization and nuclear remodeling ...
Honggang Su, Huimin Pan, Yaqiang Liu, Wei Wang, Yang Jiao, Yumo Hang, Xiahe Huang, Yong E. Zhang, Yuhang Chen, Mei Ding, Xun Huang   +10 more
wiley   +1 more source

Beyond Catalytic Therapy: Copper‐Paeonol Nanozymes Disrupt Fascin‐Mediated Actin Bundling to Suppress Tumor Growth and Metastasis

Advanced Science, EarlyView.
Copper‐paeonol nanozymes target tumor‐specific reactive oxygen species generation and disrupt fascin‐mediated actin bundling, effectively suppressing tumor growth and metastatic colonization. Abstract Fascin, an actin‐bundling protein universally upregulated in metastatic tumors, drives tumor migration and invasion by promoting filopodia and ...
Peiying Zhang, Huajun Li, Yisen Wang, Jie Xiang, Lei Fan, Shengzhe Zhang, Lizeng Gao, Hua Dai, Juqun Xi   +8 more
wiley   +1 more source

Navigating the Ethereal Tightrope: The Nanogenerator Manipulates Neurons for Immune Equilibrium

Advanced Science, EarlyView.
This review explores how nanogenerators modulate neuroimmune responses, offering innovative strategies for treating neurological disorders. By interfacing with neural pathways, they enable precise control of immune activity, especially via vagus nerve stimulation.
Jia Du, Rui Deng, Ya Wu, Hong‐Xia Ren, Zong‐Hong Lin, Yang‐Bao Miao   +5 more
wiley   +1 more source

TriCON: A Carbon‐Based Triple‐Modal Nanoplatform for Pancreatic Cancer Therapy

Advanced Science, EarlyView.
We developed TriCON, a triple‐modality nanotherapeutic platform, to treat pancreatic ductal adenocarcinoma (PDAC) by synergizing gene editing, chemotherapy, and immunotherapy. TriCON utilizes CRISPR/Cas9 to target the poliovirus receptor (PVR), combined with nano‐encapsulated doxorubicin and checkpoint blockade. This approach achieved significant tumor
Xinyu Peng, Jiaxing Huang, Shengnan Lv, Jian Zhang, Liangliang Zhang, Huan Liu, Yan Liu, Zhen‐An Qiao, Feng Wei, Bao‐Lian Su   +9 more
wiley   +1 more source

ERM Inhibition Confers Ferroptosis Resistance through ROS‐Induced NRF2 Signaling

Advanced Science, EarlyView.
ERM inhibition disrupts ERM‐actin interactions, elevating ROS and triggering KEAP1 degradation, which stabilizes and activates NRF2. Nuclear NRF2 induces cytoprotective genes, notably HMOX1, enhancing redox buffering and suppressing lipid peroxidation to resist erastin‐induced ferroptosis.
Menghao Qiao, Liqun Zhou, Minhua Zhou, Yu Fang, Haiying Mai, Lingbo Cao, Kun Xu, Yuan Sang, Minyi Chen, Jiewei Huang, Peiyi Huang, Zhipeng Yan, Chao Wang, Zhangshuai Dai, Dichun Huang, Ronghan He, Lijuan Pang, Yunmiao Guo, Ting Gang Chew, Junqi Huang   +19 more
wiley   +1 more source

HSP70 Interactome‐Mediated Proteolysis Targeting Chimera (HSP70‐PROTAC) for Ferroptosis‐Driven Cancer Treatment

Advanced Science, EarlyView.
This study reports a novel targeted protein degradation strategy termed “HSP70‐PROTAC” that recruits Hsc70 complex to a target protein for inducing degradation. Among them, GDAz‐3 exhibits effective GPX4 degradation activity via UPS/CMA processes, triggering ferroptosis‐driven anticancer activity in vitro and in vivo.
Jinyun Dong, Yulong Li, Hui Liang, Zumei Wu, Shiqun Wang, Yichao Wang, Jieyu Xu, Yanning Lan, Maohua Cai, Guangzhao Pan, Haiyan Yang, Kai Miao, Zhe‐Sheng Chen, Fangfang Tao, Xuelei Ma, Jiang‐Jiang Qin   +15 more
wiley   +1 more source

Reactivation of mTOR signaling slows neurodegeneration in a lysosomal sphingolipid storage disease

Neurobiology of Disease
Sandhoff disease, a lysosomal storage disorder, is caused by pathogenic variants in the HEXB gene, resulting in the loss of β-hexosaminidase activity and accumulation of sphingolipids including GM2 ganglioside.
Hongling Zhu, Y. Terry Lee, Colleen Byrnes, Jabili Angina, Danielle A. Springer, Galina Tuymetova, Mari Kono, Cynthia J. Tifft, Richard L. Proia   +8 more
doaj   +1 more source

APOE‐stratified Proteomic and Metabolomic Analysis Reveals Mitochondrial Dysfunction Inflammation and Lipid Dysregulation in Alzheimer's Disease

Advanced Science, EarlyView.
A large‐scale multiomic dataset (proteomic and metabolomic) comprising 3,060 plasma samples were analyzed to identify proteins, metabolites, pathways, and protein‐associated drugs linked to Alzheimer’s Disease (AD) independently of apolipoprotein E (APOE). AD was associated with a distinct molecular signature that captures.
Fuhai Li, Yike Chen, Daniel Western, Muhammad Ali, Menghan Liu, Katherine Gong, Ying Xu, Joseph Lowery, David M. Holtzman, Chloe Robins, John D. Eicher, Yen‐Ning Huang, ShiWei Liu, Tamina Park, Andrew J. Saykin, Kwangsik Nho, Mahdi Moqri, Richard C. Mohs, Amelia Farinas, Patricia Moran‐Losada, Hamilton Se‐Hwee Oh, Tony Wyss‐Coray, Carlos Cruchaga   +22 more
wiley   +1 more source

Increased Blood Flow and Tendon Swelling Precedes Vascular Expansion and Tissue Matrix Changes In Early Human Tendinopathy: A Potential Window for Superior Treatment Response

Advanced Science, EarlyView.
Human participants with early‐ or chronic‐stage tendinopathy and healthy controls are investigated using ultrasound Doppler, 3T and 7T MRI, and tendon biopsies. Tendon swelling and hyperperfusion are evident early, while vascular growth and extracellular matrix changes are most pronounced in chronic tendinopathy. Early intervention may provide superior
Max F. R. Merkel, Nikolaj M. Malmgaard‐Clausen, Marius Lendal, Hartwig R. Siebner, René B. Svensson, Stephanie G. Dakin, Marcus Krüger, Luisa Schmidt, Jakob Agergaard, Ching‐Yan Chloé Yeung, S. Peter Magnusson, Michael Kjaer   +11 more
wiley   +1 more source