Results 111 to 120 of about 40,385 (227)

Lysosomal Storage Diseases: Is Impaired Apoptosis a Pathogenic Mechanism?*

, 2004
International audienceLysosomal storage disorders are inborn diseases resulting from the lack or deficient activity of lysosomal hydrolases, transporters, or integral membrane proteins.
Tardy, Claudine, Andrieu-Abadie, Nathalie, Salvayre, Robert, Levade, Thierry   +3 more
core   +1 more source

Targeting the HSPA8‐CMA‐ATP6V1A Axis Triggers Lysosomal Hyperacidification and Catastrophic Vacuolation in Prostate Cancer

Advanced Science, EarlyView.
Our experimental evidence supports a model in which ALO targets the HSPA8‐CMA‐ATP6V1A axis to induce lysosomal hyperacidification and initiate osmotic and lipidomic stress. These changes are associated with LMP and loss of lysosomal integrity in prostate cancer cells.
Bingzheng An, Ze Gao, Shuo Chen, Liwei Meng, Chen Zhang, Kefan Song, Haochen Cui, Lei Yan, Zhiqing Fang   +8 more
wiley   +1 more source

Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.

, 2002
Paediatric neurodegenerative diseases are frequently caused by inborn errors in glycosphingolipid (GSL) catabolism and are collectively termed the glycosphingolipidoses.
Platt, Frances, Jeyakumar, M, M. Jeyakumar, Platt, FM, R. A. Dwek, F. M. Platt, Dwek, RA, T. D. Butters, Butters, TD   +8 more
core   +1 more source

A Metal‐Phenolic Nanocluster Orchestrates Mito‐Ca2+ Metabolic Autonomy for Tumor Ca2+ Interference Therapy

Advanced Science, EarlyView.
Tumor Ca2+ interference therapy suffers from self‐protective Ca2+ metabolic autoregulation. In this scenario, a versatile metal‐phenolic nanocluster (TCMH) is engineered to modulate mitochondrial calcium uniporter (MCU) ‐mediated mito‐Ca2+ metabolic autonomy.
Ronglong Chen, Jia Huang, Yuepeng Wang, Shucong Yao, Fei Wu, Juan Liu, Chunxue Song, Li Lin, Xijun Lin, Yingjie Deng, Chao Zhang, Zhiquan Huang, Zixian Huang, Lisi Xie   +13 more
wiley   +1 more source

Mechanically Regulated Nanozymes for Remote Metabolic Reprogramming and Precise Cancer Therapy

Angewandte Chemie, EarlyView.
Inspired by mechanoenzymes, a ferrocene‐based mechanically regulated nanozyme converts acoustic shear force into enhanced peroxidase‐like activity by decreasing electron density and alleviating steric hindrance at the Fe active sites, thereby rewiring redox homeostasis. Leveraging this remote regulatory mechanism, a multienzyme‐regulated nanoreactor is
Fangman Chen, Xiaochun Xie, Hanyao Huang, Ka Hong Wong, Jiying Liu, Hui Fang, Shaowen Wang, Jianfang Cao, Yu Tao, Mingqiang Li, Chao Yang, Wen Sun, Dan Shao, Yunlu Dai   +13 more
wiley   +2 more sources

Perinatal Gene Transfer to the Liver

, 2011
The liver acts as a host to many functions hence raising the possibility that any one may be compromised by a single gene defect. Inherited or de novo mutations in these genes may result in relatively mild diseases or be so devastating that death within
Buckley, SM, Ward, N.J., Buckley, S.M.K., Ward, NJ, Chan, J.K.Y., Waddington, S.N., Rahim, A.A., Waddington, SN, Rahim, AA, Chan, JK, Mckay, T.R., Howe, S.J., Howe, SJ, McKay, TR   +13 more
core   +1 more source

Material‐Encoded Synchronization of Immunogenic Cell Death With Adenosine A2A Receptor Blockade Reprograms the Tumor Microenvironment

Advanced Science, EarlyView.
A bismuth–copper diselenide–based nanoplatform (BSCS@PHY) coordinates immunogenic cell death with local A2A receptor blockade in 4T1 tumors. Thermally triggered shell melting exposes catalytic surfaces for glutathione depletion and chemodynamic ROS generation, while co‐delivering an A2AR antagonist and yeast‐wall adjuvant to enhance dendritic‐cell ...
Xiangting Yi, Hanlou Yang, Junting Huang, Pengfei Wu, Shiying Zhou, Zunde Liao, Min Han, Zishan Chen, Xiaoyu Huang, Nan Ma, Jie Zhou, Mingqiang Li, Yiling Zhong   +12 more
wiley   +1 more source

TFEB overexpression alleviates autophagy-lysosomal deficits caused by progranulin insufficiency

Scientific Reports
Progranulin is a pro-protein that is necessary for maintaining lysosomal function. Loss-of-function progranulin (GRN) mutations are a dominant cause of frontotemporal dementia (FTD).
Wren O. Nader, Kaylan S. Brown, Nicholas R. Boyle, Azariah K. Kaplelach, Shaimaa M. Abdelaziz, Skylar E. Davis, Qays Aljabi, Ahmad R. Hakim, Amelia G. Davidson, Giacynta A. Vollmer, Leah C. Wright, J. Bailey Echols, Joelle Saad, Nicholas S. Pena, Andrew E. Arrant   +14 more
doaj   +1 more source

Porcine Deltacoronavirus M Protein Binds NLRP3 to Promote Inflammasome Assembly via Competition with TRIM31

Advanced Science, EarlyView.
Porcine deltacoronavirus (PDCoV) infection induces severe intestinal inflammation and acute diarrhea in piglets, yet the molecular mechanism remains incompletely understood. The M protein activates NLRP3 inflammasome through dual mechanisms: direct binding to the NLRP3 LRR domain and disruption of TRIM31‐mediated K48‐linked ubiquitination.
Jinhui Hou, Fangfang Han, Anqi Liu, Yang Yu, Rui Zhao, Zhengting Shi, Yanrong Lv, Jinli Liu, Shaopo Zu, Zhanyong Wei, Jin Yuan, Hui Hu   +11 more
wiley   +1 more source

Curated incidence of lysosomal storage diseases from the Taiwan Biobank. [PDF]

NPJ Genom Med, 2023
Tsai MM, Hung MZ, Lin YL, Lee NC, Chien YH, Hwu WL.   +5 more
europepmc   +1 more source