Results 91 to 100 of about 31,581 (280)

Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders

Molecular Genetics and Metabolism Reports, 2018
Totally implantable vascular access devices (TIVADs) are commonly used in conjunction with enzyme replacement therapy (ERT) for lysosomal storage disorders (LSDs).
Christian J. Hendriksz, Paul Harmatz, Roberto Giugliani, Jane Roberts, G. Suren Arul   +4 more
doaj   +1 more source

Cholesterol Laundry of Cell Membrane and Fatty Liver by Detergent Liposomes to Improve Anti‐Cancer Drug Responsiveness of Patient Liver Tissues

Advanced Science, EarlyView.
Cholesterol‐enriched plasma membranes in hepatocellular carcinoma impede drug penetration. Cholesterol (+)‐liposomes act as membrane‐specific detergents, extracting cholesterol and reducing barrier function without cytotoxicity. Following endocytosis, cholesterol transfers from endosomes to liposomes and is metabolized in the ER.
Chansik Kim, Joo Kyung Noh, Sewoom Baek, Seung Eun Yu, Jueun Kim, Seongyo Lee, Youngji Oh, Dai Hoon Han, Seyong Chung, Hak‐Joon Sung   +9 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

Newborn Screening for Lysosomal Storage Disorders in Belgium

Journal of Inborn Errors of Metabolism and Screening, 2017
Lysosomal storage disorders (LSDs) are a group of metabolic disorders with various clinical presentations, which complicate diagnosis. A pilot study was performed to test the appropriateness and effectiveness of the newborn screening method for Pompe ...
Francois Eyskens MD, PhD, Sylvie Devos PhD   +1 more
doaj   +1 more source

Characterization and downstream mannose phosphorylation of human recombinant α-L-iduronidase produced in Arabidopsis complex glycan-deficient (cgl) seeds [PDF]

, 2013
This work was supported by a Wellcome Trust award to TMG.Mucopolysaccharidosis (MPS) I is a lysosomal storage disease caused by a deficiency of α-L-iduronidase (IDUA) (EC 3.2.1.76); enzyme replacement therapy is the conventional treatment for this ...
Brooks, Doug, Gloster, Tracey M., Vocadlo, D.J., Brooks, D., Kolarich, Daniel, Kermode, Allison R, Kermode, Allison R., Haselhorst, T., Qian, Y., Gloster, Tracey M, Qian, Yi, Gloster, Tracey, Kolarich, D., Haselhorst, Thomas, Packer, N.H., Pierce, Owen, He, X., von Itzstein, Mark, Gloster, T.M., Packer, Nicolle H., He, Xu, Pierce, O., Packer, Nicolle H, Vocadlo, David J, von Itzstein, M., Kermode, A.R., Vocadlo, David J.   +26 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

Inhibition of PIKfyve Leads to Lysosomal Disorders via Dysregulation of mTOR Signaling

Cells
PIKfyve is an endosomal lipid kinase that synthesizes phosphatidylinositol 3,5-biphosphate from phosphatidylinositol 3-phsphate. Inhibition of PIKfyve activity leads to lysosomal enlargement and cytoplasmic vacuolation, attributed to impaired lysosomal ...
Jianhong Xia, Haiyun Wang, Zhihang Zhong, Jun Jiang   +3 more
doaj   +1 more source

Lysosomal storage diseases as disorders of autophagy

, 2008
The cellular turnover of proteins and organelles requires cooperation between the autophagic and the lysosomal degradation pathways. A crucial step in this process is the fusion of the autophagosome with the lysosome.
Rubinsztein D.C., BALLABIO, ANDREA, FRALDI, ALESSANDRO, SETTEMBRE, CARMINE   +3 more
core  

Biomimetic Macrophage Cell Membrane‐Based Nanoparticles for Effective Treatment of Glioblastoma Through Boron Neutron Capture Therapy Combined With Immunotherapy

Advanced Science, EarlyView.
ABSTRACT Glioblastoma multiforme (GBM) remains largely incurable due to the blood‐brain barrier (BBB) and immunosuppressive microenvironment. While boron neutron capture therapy (BNCT) selectively eradicates tumor cells via 10B(n, α)‐7Li reactions, its clinical potential in GBM is unrealized because of the suboptimal pharmacokinetics of conventional ...
Jiawen Chen, Haoyu You, Hefa Huang, Huihui Chai, Ruize Zhu, Yun Guan, Qisheng Tang, Tianwen Li, Shan Jiang, Houshi Xu, Peng Wang, Yue Wang, Maoyuan Sun, Beining Liu, Zhen Li, Yulai Zeng, Weiqiu Ping, Yanlin Teng, Songlin Yan, Qinghui Li, Long Gu, Tao Sun, Zhifeng Shi   +22 more
wiley   +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