Results 111 to 120 of about 42,400 (289)

Functional screening of lysosomal storage disorder genes identifies modifiers of alpha-synuclein neurotoxicity. [PDF]

PLoS Genet, 2023
Yu M, Ye H, De-Paula RB, Mangleburg CG, Wu T, Lee TV, Li Y, Duong D, Phillips B, Cruchaga C, Allen GI, Seyfried NT, Al-Ramahi I, Botas J, Shulman JM.   +14 more
europepmc   +1 more source

Dual Physiological Barriers Bypassed by a Silk‐Based Supramolecular Protein Delivery Platform for Neuroinflammation Mitigation in Alzheimer's Disease

Advanced Science, EarlyView.
A supramolecular nanocomplex based on phenolic‐modified silk sericin is developed to deliver antioxidant enzymes across the blood–brain barrier and escape lysosomal degradation. This dual‐barrier‐crossing system reduces neuroinflammation and improves cognitive performance in Alzheimer's disease mouse model, offering a promising strategy for protein ...
Doudou Hu, Tiandong Li, Jian He, Yujian Jiang, Yeyuan Wang, Jingchen Sun   +5 more
wiley   +1 more source

Multiplexing Iduronate-2-Sulphatase (MPS-II) into a 7-Plex Lysosomal Storage Disorder MS/MS Assay Using Cold-Induced Phase Separation. [PDF]

Int J Neonatal Screen, 2023
Courtney E, Pickens CA, Cuthbert C, Petritis K.   +3 more
europepmc   +1 more source

Decoding Undesirable Inflammatory Responses of Nucleic Acid‐Delivering Lipid Nanoparticles

Advanced Science, EarlyView.
Lipid nanoparticles (LNPs) enable efficient nucleic acid delivery, but their immunogenicity is a double‐edged sword. This review explores LNP‐driven innate and adaptive immunity, covering lipid components, endosomal escape, and nucleic acid sensing.
Ruimin Hu, Yin Dou, Chenwen Li, Yuying Mu, Huiying Ouyang, Wenhao Shen, Jianxiang Zhang   +6 more
wiley   +1 more source

Bottom‐Up Coacervate‐Based Artificial Cells: Integrating Cellular Hallmarks into Complex Life‐Like Systems

Angewandte Chemie, EarlyView.
Current interest in artificial cell research underscores its potential to deepen our understanding of life's fundamental processes. This review highlights advances in bottom‐up coacervate‐based artificial cell engineering via combined integration of cellular hallmarks.
Arjan Hazegh Nikroo, Angshuman Das, Madelief A. M. Verwiel, Jan C. M. van Hest   +3 more
wiley   +2 more sources

Extracellular Vesicles Released by Genetically Modified Macrophages Activate Autophagy and Produce Potent Neuroprotection in Mouse Model of Lysosomal Storage Disorder, Batten Disease. [PDF]

Cells, 2023
El-Hage N, Haney MJ, Zhao Y, Rodriguez M, Wu Z, Liu M, Swain CJ, Yuan H, Batrakova EV.   +8 more
europepmc   +1 more source

Lysosomal storage disorders: Present and future [PDF]

Indian Pediatrics, 2015
ysosomal storage disorders (LSDs) is a group of more than 50 single gene disorders caused by deficiencies of any of the glycoprotein acid hydrolase enzymes. Lysosomes are intracellular organelles serving important functions of breakdown of large molecules and helping in their disposal.
openaire   +2 more sources

ZDHHC18‐Mediated Palmitoylation of ORF3a Promotes SARS‐CoV‐2 Pathogenesis by Antagonizing TRIM16‐Mediated Ubiquitination and Proteasomal Degradation

Advanced Science, EarlyView.
Palmitoylation by ZDHHC18 blocks ORF3a K27‐linked ubiquitination mediated by TRIM16, thereby preventing its proteasomal degradation and strengthening viral pathogenesis. Targeting palmitoylation through a pharmacological inhibitor (2‐BP), a competitive inhibitory peptide (OPIP), or adenovirus‐mediated knockdown of ZDHHC18 expression presents a ...
Sidi Yang, Kun Li, Lihong Liu, Linsen Zeng, Qifeng Deng, Jiacheng Huang, Xiaoran Dong, Xin Wang, Jianwei Liang, Hongchao Liu, Hong Peng, Yuxin Lin, Xiaolu Xie, Yuzhen Ye, Tiefeng Xu, Zhaohuan Wang, Chun‐Mei Li, Deyin Guo   +17 more
wiley   +1 more source

The Association Between Lysosomal Storage Disorder Genes and Parkinson's Disease: A Large Cohort Study in Chinese Mainland Population. [PDF]

Front Aging Neurosci, 2021
Zhao YW, Pan HX, Liu Z, Wang Y, Zeng Q, Fang ZH, Luo TF, Xu K, Wang Z, Zhou X, He R, Li B, Zhao G, Xu Q, Sun QY, Yan XX, Tan JQ, Li JC, Guo JF, Tang BS.   +19 more
europepmc   +1 more source

Glycogenosis type II : a study on clinical heterogeneity and enzyme replacement therapy [PDF]

, 1989
Glycogenosis type II is a lysosomal storage disorder caused by deficiency of acid a-glucosidase and characterized by heart failure and skeletal muscle weakness.
Ploeg, A.T. (Ans) van der
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