Results 71 to 80 of about 42,400 (289)

Targeting the PGRN‐BMP Lysosomal Axis With NPs@PGRN Reverses Immunometabolic Dysfunction in Chronic Septic Arthritis

Advanced Science, EarlyView.
Chronic septic arthritis involves intracellular bacterial persistence and lipid‐immune crosstalk via the PGRN‐BMP lysosomal axis. A dual‐targeting nanoparticle system (NPs@PGRN) restores lysosomal bactericidal function, reduces bacterial burden, and reprograms macrophage immunity, offering a novel therapeutic strategy. ABSTRACT Chronic septic arthritis,
Congsun Li, Jiaqi Fan, Tao Sun, Wushi Cui, Jiaxuan Zou, Weinan Yang, Liang Chen, Jian Xiao, Shicheng Wang, Tao Zhang, Jianqiao Hong, An Liu, Haobo Wu   +12 more
wiley   +1 more source

A hopeful therapy for Niemann-Pick C diseases [PDF]

, 2017
Not abstract ...
Erickson, Robert P., Fiorenza, Maria Teresa   +1 more
core   +1 more source

Unraveling the Molecular Mechanisms Underlying Spontaneous Multipolar Mitosis Through CIN‐seq

Advanced Science, EarlyView.
Multipolar mitosis, a hallmark of chromosomal instability (CIN), drives tumor heterogeneity but is challenging to study in live cells. Using CIN‐seq, a single‐cell multiomics method, we profiled rare CIN events and identified mechanisms associated with viable multipolar mitosis, including PTEN attenuation, Rho GTPase‐driven cytokinesis failure, and ...
Pin‐Rui Su, Ting‐Chun Chou, Maria Teresa López‐Cascales, Junfeng Huang, Tsai‐Ying Chen, Sjoukje van Wieren, Chan Li, Cecile Beerens, Li You, Jelle Storteboom, Miao‐Ping Chien   +10 more
wiley   +1 more source

Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy

Orphanet Journal of Rare Diseases, 2022
Background Fabry disease (FD) is a treatable X-linked condition leading to progressive cardiac disease, arrhythmia and premature death. We aimed to increase awareness of the arrhythmogenicity of Fabry cardiomyopathy, by comparing device usage in patients
Ravi Vijapurapu, William Bradlow, Francisco Leyva, James C. Moon, Abbasin Zegard, Nigel Lewis, D. Kotecha, Ana Jovanovic, Derralynn A. Hughes, Peter Woolfson, Richard P. Steeds, Tarekegn Geberhiwot   +11 more
doaj   +1 more source

A Nanoparticle‐Integrated Complete Manufacturing Pipeline of Chemically Engineered Exosomes

Advanced Science, EarlyView.
We report a novel manufacture technology of chemically engineered exosomes. The four steps of manufacturing, i.e., biogenesis, loading, isolation, and storage, are integrated by the use of a nanoparticle. The manufacture technology incorporates three innovative components, i.e., a new nano‐bio effect, a new composite nanoparticle, and a new isolation ...
Xiaowei Wen, Zixing Xu, Zerun Hao, Yanming Chen, Kai Xie, Haofan Yin, Xueying Wang, Jie Min, Sihan Sun, Baiding Chen, Chengxiu Ling, Mingming Xu, Yizhao Chen, Gang Ruan   +13 more
wiley   +1 more source

Inappropriate cathepsin K secretion promotes its enzymatic activation driving heart and valve malformation

JCI Insight, 2020
Although congenital heart defects (CHDs) represent the most common birth defect, a comprehensive understanding of disease etiology remains unknown.
Po-Nien Lu, Trevor Moreland, Courtney J. Christian, Troy C. Lund, Richard A. Steet, Heather Flanagan-Steet   +5 more
doaj   +1 more source

Hierarchical Targeting of TREM2+ Myeloid Cells via Acid‐Triggered OMVs Reprogram Immunosuppression and Suppress Osteolysis in Bone‐Metastatic TNBC

Advanced Science, EarlyView.
This study developed a hierarchical targeting nanoplatform, siTREM2@ETP‐PEOz‐OMVs, against triple‐negative breast cancer (TNBC) bone metastasis. It precisely delivers therapeutic siTREM2 to monocytes/macrophages within the metastatic niche. This intervention dually regulates cell fate: reprogramming immunosuppressive macrophages and inhibiting ...
Fanglu Chen, Yucheng Xue, Xiao Ma, Liang Chen, Zhenxuan Shao, Hangxiang Sun, Haochen Mou, Fangqian Wang, Zilong Li, Yechao Shen, Zhaoming Ye, Xiaohua Yu, Shengdong Wang   +12 more
wiley   +1 more source

Oral Migalastat HCl Leads to Greater Systemic Exposure and Tissue Levels of Active α-Galactosidase A in Fabry Patients when Co-Administered with Infused Agalsidase. [PDF]

, 2015
UnlabelledMigalastat HCl (AT1001, 1-Deoxygalactonojirimycin) is an investigational pharmacological chaperone for the treatment of α-galactosidase A (α-Gal A) deficiency, which leads to Fabry disease, an X-linked, lysosomal storage disorder. The currently
Adera, Mathews, Barlow, Carrolee, Barth, Jay, Bichet, Daniel G, Boudes, Pol, Eyskens, Francois, Flanagan, John J, Goker-Alpan, Ozlem, Holida, Myrl, Johnson, Franklin K, Khanna, Richie, Lockhart, David J, Nicholls, Kathy, Shankar, Suma, Sitaraman, Sheela, Thomas, Mark, Valenzano, Kenneth J, Warnock, David G, Wustman, Brandon A   +18 more
core   +4 more sources

Tetrastigma Hemsleyanum Polysaccharide Suppresses Triple‐Negative Breast Cancer by Disrupting the Hippo‐YAP/TEAD4‐PDIA4 Axis and Endoplasmic Reticulum Stress Adaptation

Advanced Science, EarlyView.
ABSTRACT Triple‐negative breast cancer (TNBC) exhibits addiction to chronic endoplasmic reticulum (ER) stress, which sustains an aggressive metastatic phenotype through activation of the unfolded protein response (UPR). Here, we identify a previously unrecognized “ER‐stress addiction” axis in which the Hippo pathway effector TEAD4 directly ...
Yini Shang, Wentao Si, Youxue Zhang, Jialin Liu, Huaixi Zhang, Yafei Li, Hongmeng Su, Wei Jiang, Zhishan Ding, Lihong Wang   +9 more
wiley   +1 more source

Generation and characterization of two iPSC lines derived from subjects with Free Sialic Acid Storage Disorder (FSASD)

Stem Cell Research
Free sialic acid storage disorder (FSASD) is a rare, autosomal recessive, neurodegenerative disorder caused by biallelic mutations in SLC17A5, encoding the lysosomal transmembrane sialic acid exporter, SLC17A5.
Marya S. Sabir, Petcharat Leoyklang, Mary E. Hackbarth, Evgenia Pak, Amalia Dutra, Richard Tait, Laura Pollard, David R. Adams, William A. Gahl, Marjan Huizing, May Christine V. Malicdan   +10 more
doaj   +1 more source