Results 151 to 160 of about 154,911 (267)

Nucleoside‐Modified mRNA Encoding Alpha‐Galactosidase A Ameliorates Fabry Disease Phenotypes in Human IPSC‐Derived Cardiomyocytes

Advanced Science, EarlyView.
Human iPSC‐derived Fabry cardiomyocytes exhibited broad transcriptional dysregulation, apoptosis, mitochondrial dysfunction, impaired reactive oxygen species handling, altered contractility, and abnormal calcium transient decay, potentially mediated by phospholamban hyperphosphorylation.
Malte Juchem, Lea Oehlsen, Sedef Ersoy, Jia Li Ye, Natalie Weber, Elisa Mohr, Wilson Agyapong, Junqing Liu, Maximilian Fuchs, Ke Xiao, Christopher Jahn, Reto Eggenschwiler, Tobias Cantz, Julia Beimdiek, Falk F. R. Buettner, Theresia Kraft, Jan Hegermann, Ivonne J. Knorr, Lisa Ernst, Linda Feldbrügge, Resa Puffert, Kevin Schmidt, Christian Bär, Jeannine Hoepfner, Thomas Thum   +24 more
wiley   +1 more source

Autophagy and Mitophagy in Hypertensive Chronic Kidney Disease: Evidence Grading, Cell-Type Divergence, and a Working Lysosomal Hypothesis. [PDF]

Life (Basel)
Han S, Chang YK, Jo J, Choi DE.
europepmc   +1 more source

USP30‐Mediated Deubiquitination of PEX5 Suppresses Pexophagy to Drive Tubular Injury in Diabetic Kidney Disease

Advanced Science, EarlyView.
USP30 accumulation in diabetic renal tubular epithelial cells deubiquitinates PEX5, suppresses pexophagy, and drives dysfunctional peroxisome accumulation and tubular injury. USP30 depletion restores PEX5 ubiquitination and pexophagy, improves peroxisomal function, and alleviates tubular damage, highlighting USP30 as a therapeutic target in diabetic ...
Jia Li, Chaoyang Hua, Guangpu Li, Furong Zhai, Jingjing Ren, Shaokang Pan, Qi Feng, Jianguo Wen, Dongwei Liu, Zhangsuo Liu, Jiayu Duan   +10 more
wiley   +1 more source

A HILIC-MS/MS method for simultaneous quantification of the lysosomal disease markers galactosylsphingosine and glucosylsphingosine in mouse serum. [PDF]

Biomed Chromatogr, 2018
Sidhu R, Mikulka CR, Fujiwara H, Sands MS, Schaffer JE, Ory DS, Jiang X.   +6 more
europepmc   +1 more source

A Decoy‐Receptor‐Armed Biomimetic Nanotherapeutic With Inherent Tropism for Conserved Pathogenic Macrophages for Treating Osteoarthritis and Intervertebral Disc Degeneration

Advanced Science, EarlyView.
A biomimetic doppelgänger nanosystem neutralizes extracellular inflammatory cytokines and silences intracellular pyroptosis, reprogramming pathogenic macrophages to attenuate both joint and spine degeneration. ABSTRACT Osteoarthritis (OA) and intervertebral disc degeneration (IVDD) are debilitating musculoskeletal disorders driven by shared ...
Fudong Li, Bin Zhang, Zhiqiu Zhang, Bing Zheng, Linhui Han, Chen Yan, Jialin Li, Kaiqiang Sun, Tianyi Zhao, Jiangang Shi   +9 more
wiley   +1 more source

TLDc proteins: Stress-responsive nexus regulators of redox signaling, V-ATPase dynamics, and organellar crosstalk in neurodegeneration. [PDF]

Redox Biol
Ghufran MS, Soni P, Thomas B.
europepmc   +1 more source

SR‐A3 Promotes USP18‐Mediated deISGylation of STING to Protect Against Myocardial Ischemia‐Reperfusion Injury

Advanced Science, EarlyView.
MI/RI is a major clinical challenge with high morbidity and mortality. This study demonstrates that SR‐A3 expression is downregulated in MI/RI, and its deletion exacerbates damage via the USP18/ISG15/STING axis, revealing SR‐A3 as a potential therapeutic target.
Yufei Han, Guolin Miao, Yinqi Zhao, Zhiyong Du, Kaikai Lu, Changqi Xiao, Aiden Xian, Jiaxin Duanmu, Zihao Zhou, Jinxuan Chen, Lianxin Zhang, Chao Zhai, Yitong Xu, Zhaoling Li, Xianqin Shen, Sijing Shi, Jingxuan Chen, Si Mei, Yuan Fang, Liwen Zheng, Ling Zhang, Yuhui Wang, Wei Huang, Erdan Dong, Xunde Xian   +24 more
wiley   +1 more source

LRRK2: Molecular Mechanisms in Parkinson's Disease. [PDF]

Int J Mol Sci
Arias-Carrión O, Guerra-Crespo M, Ortuño-Sahagún D, Ortega-Robles E.   +3 more
europepmc   +1 more source

A New Recruitable E3 Ligase UHRF1 Supporting Targeted Protein Degradation: A Minimal Azide as a Recruitment Ligand

Advanced Science, EarlyView.
Targeted protein degradation is often limited by the scarcity of usable E3 ligases. Herein, we report the first small‐molecule GPX4 degraders that incorporate either electrophilic warheads or a minimal azide group as an E3 recruitment ligand. The azide‐based degrader DK‐5070 effectively drives potent GPX4 degradation; mechanistic studies reveal that ...
Zehong Lin, Kang Duan, Rui Wan, Tao Jiang, Jinzhe Wei, Miao Sun, Mariia A. Skryl'nikova, Maxim A. Gureev, Zhi‐Min Zhang, Zhang Zhang, Ke Ding, Nan Ma, Tongzheng Liu, Yi Tan, Zhengqiu Li   +14 more
wiley   +1 more source

Trehalose's untapped mechanisms in alzheimer's: gut-brain-autophagy signalling beyond the usual targets. [PDF]

Daru
Gunasekaran SK, K HKC, Gobinath M, Roychowdhury P, N MS.   +4 more
europepmc   +1 more source