Results 61 to 70 of about 114,325,321 (319)

Human Urine Stem Cells Alleviate Pulmonary Fibrosis via Inhibiting Macrophage‐Myofibroblast Transition

Advanced Science, EarlyView.
Therapeutic role and mechanism of human urine stem cells (hUSCs) in pulmonary fibrosis. hUSCs alleviated pulmonary fibrosis by selectively inhibiting macrophage‐myofibroblast transition (MMT) in two ways: on one hand, hUSCs inhibited mitochondrial reactive oxygen species (mtROS) production and apoptosis/senescence of epithelial cells in pulmonary ...
Zhou‐Hang Zhang, Guan‐Lin Guo, Xiao‐Hui Guan, Min Hu, Qi‐Ming Huang, Ding‐Wen Guo, Hao‐Cheng Gu, You‐Qiong Zhuo, Ning Li, Hong‐Bo Xin, Ke‐Yu Deng   +10 more
wiley   +1 more source

Targeting NSUN2‐Mediated m5C Modification Attenuates Chondrocyte Senescence and NLRP3 Activation in Osteoarthritis

Advanced Science, EarlyView.
NSUN2‐mediated m5C modification cooperates with ALYREF to stabilize and export IP3R3 mRNA, increasing IP3R3 expression and Ca2 + overload in chondrocytes. This signaling promotes mitochondrial dysfunction, NLRP3 inflammasome activation, and senescence, thereby accelerating osteoarthritis progression.
Guping Mao, Wei Li, Zhencan Lin, Zengfa Deng, Ming Li, Zongrui Jiang, Changzhao Li, Dianbo Long, Yan Kang   +8 more
wiley   +1 more source

HMGCR‐Driven Cholesterol Metabolism Promotes Osteoarthritis Progression by Accelerating Synovial Fibroblast Senescence

Advanced Science, EarlyView.
In the pathological context of osteoarthritis (OA), the phosphorylation of AKT1 at Ser473 enhances its binding to Lys140 of Insig1, which facilitates the formation of AKT1–Insig1 complex. Subsequently, the activation of AKT1 promotes the phosphorylation of Insig1 at Ser189, potentially enhancing the dissociation of Insig1 from sterol regulatory element‑
Xiaoqi Zhang, Siru Zhou, Xi Liu, Wei Xiang, Xiuqin Peng, Yu Tian, Song Li, Hailin Wang, Bo Liao, Shunzheng Fang, Runze Jin, Jinhui Wu, Chuanqing Bai, Wei Zhang, Bo Huang, Bingfei Li, Lifeng Chen, Lin Chen, Bin Zhang, Zhenhong Ni   +19 more
wiley   +1 more source

A Novel Mutation in The GLA Gene Leading to Fabry Disease - A Case Report from Islamabad, Pakistan

Life and Science
Fabry disease (OMIM #301500) is a rare X-linked lysosomal storage disease. Generally, lysosomal storage disease is identified by inappropriate lipid storage in lysosomes due to specific enzyme deficiencies.
Ayesha Ali Malick, Muhammad Jawad Hassan, Arsalan Ahmad   +2 more
doaj   +1 more source

Consensus recommendations for the treatment and management of patients with Fabry disease on migalastat: a modified Delphi study

Frontiers in Medicine, 2023
ObjectiveFabry disease is a progressive disorder caused by deficiency of the α-galactosidase A enzyme (α-Gal A), leading to multisystemic organ damage with heterogenous clinical presentation.
Daniel G. Bichet, Robert J. Hopkin, Patrício Aguiar, Patrício Aguiar, Sridhar R. Allam, Sridhar R. Allam, Yin-Hsiu Chien, Yin-Hsiu Chien, Roberto Giugliani, Roberto Giugliani, Staci Kallish, Sabina Kineen, Olivier Lidove, Olivier Lidove, Dau-Ming Niu, Dau-Ming Niu, Iacopo Olivotto, Juan Politei, Paul Rakoski, Roser Torra, Camilla Tøndel, Camilla Tøndel, Derralynn A. Hughes   +22 more
doaj   +1 more source

TPI1 Loss Triggers a Metabolite‐Driven Mitochondrial Redox Vulnerability via the SARM1–cADPR–Ca2+ Axis

Advanced Science, EarlyView.
Metabolite‐driven redox stress governs cancer cell senescence. TPI1 deficiency elevates DHAP, initiating SARM1‐dependent cADPR‐Ca2+ release. Mitochondrial ROS surge subsequently induces DNA damage and senescence, offering a conserved therapeutic target in multiple cancers.
Chunyu Liu, Shun Wu, Chuang Wang, Zirui Zhou, Tianwei Cai, Wen Tao, Shidong Zuo, Chi Zhang, Yuhao Dong, Yi Feng, Qingbo Huang, Baojun Wang, Xin Ma, Haoli Ma, Xu Zhang, Yan Huang   +15 more
wiley   +1 more source

Genetically Modified Cell Transplantation Through Macroencapsulated Spheroids with Scaffolds to Treat Fabry Disease

Cell Transplantation, 2021
Cell transplantation is expected to be another strategy to treat lysosomal diseases, having several advantages compared to enzyme replacement therapy, such as continuous enzyme secretion and one-time treatment to cure diseases.
Daisuke Kami, Yosuke Suzuki, Masashi Yamanami, Takahiro Tsukimura, Tadayasu Togawa, Hitoshi Sakuraba, Satoshi Gojo   +6 more
doaj   +1 more source

Renal Clearable Luminogenic Reporter for Ultrasensitive Influenza Virus Imaging and Efficient Antiviral Therapies Monitoring in Living Mice

Advanced Science, EarlyView.
A viral luminogenic reporter with chemiluminescence/fluorescence dual‐response and efficient renal clearance was developed for non‐invasive imaging and urinalysis of H1N1 virus protease, allowing for remote detection of H1N1 virus infections and monitoring of antiviral therapeutic efficacy in living mice.
Bankang Ruan, Chudan Liang, Meilin Hu, Weiping Xu, Jingjing Che, Jun Qian, Bingcai Jiang, Linna Liu, Xiancai Ma, Jiaguo Huang   +9 more
wiley   +1 more source

Rhenium‐Based β‐Galactosidase Probe Allows Senescence Profiling by Mass Cytometry

Angewandte Chemie, EarlyView.
Senescence‐associated β‐galactosidase (SA‐β‐Gal) activity is a widely accepted biomarker of cellular senescence, yet its integration with other senescence markers has been limited by available detection methods. Here, we introduce ReGal, a mass cytometry‐compatible SA‐β‐Gal activity probe that can be multiplexed with established markers of senescence ...
Adam Tam, Kris Elbein, Aleida G. Gonzalez, Edgar A. Arriaga, Mark Nitz   +4 more
wiley   +2 more sources

AAV2 vector harboring a liver-restricted promoter facilitates sustained expression of therapeutic levels of alpha-galactosidase A and the induction of immune tolerance in Fabry mice.

Molecular Therapy, 2004
The successful application of gene therapy for the treatment of genetic diseases such as Fabry is reliant on the development of vectors that are safe and that facilitate sustained expression of therapeutic levels of the transgene product. Here, we report
Robin J. Ziegler, S. Lonning, D. Armentano, Chester Li, David W. Souza, Maribeth Cherry, Christine Ford, Christine M. Barbon, R. Desnick, G. Gao, James M. Wilson, R. Peluso, Simon G. Godwin, B. Carter, R. Gregory, S. Wadsworth, Seng H. Cheng   +16 more
semanticscholar   +1 more source