Results 41 to 50 of about 14,087 (142)

Synthesis and Application of Bioactive N‐Functionalized Aziridines

Angewandte Chemie, Volume 137, Issue 44, October 27, 2025.
This review discusses modern synthetic methods for the preparation of aziridine‐containing small molecules, including biocatalytic, electrocatalytic, and photocatalytic strategies. We highlight the compatibility of various synthetic methods with control of the exocyclic N‐substituent.
Hao Tan, Samya Samanta, Nan Qiu, Alexander Adibekian, David C. Powers   +4 more
wiley   +2 more sources

Visualization of Active Glucocerebrosidase in Rodent Brain with High Spatial Resolution following In Situ Labeling with Fluorescent Activity Based Probes.

PLoS ONE, 2015
Gaucher disease is characterized by lysosomal accumulation of glucosylceramide due to deficient activity of lysosomal glucocerebrosidase (GBA). In cells, glucosylceramide is also degraded outside lysosomes by the enzyme glucosylceramidase 2 (GBA2) of ...
Daniela Herrera Moro Chao, Wouter W Kallemeijn, Andre R A Marques, Marie Orre, Roelof Ottenhoff, Cindy van Roomen, Ewout Foppen, Maria C Renner, Martina Moeton, Marco van Eijk, Rolf G Boot, Willem Kamphuis, Elly M Hol, Jan Aten, Hermen S Overkleeft, Andries Kalsbeek, Johannes M F G Aerts   +16 more
doaj   +1 more source

Ambroxol Hydrochloride Improves Motor Functions and Extends Survival in a Mouse Model of Familial Amyotrophic Lateral Sclerosis

Frontiers in Pharmacology, 2019
Amyotrophic lateral sclerosis (ALS) is a multifactorial and fatal neurodegenerative disease. Growing evidence connects sphingolipid metabolism to the pathophysiology of ALS.
Alexandra Bouscary, Alexandra Bouscary, Cyril Quessada, Cyril Quessada, Althéa Mosbach, Althéa Mosbach, Noëlle Callizot, Michael Spedding, Jean-Philippe Loeffler, Jean-Philippe Loeffler, Alexandre Henriques, Alexandre Henriques, Alexandre Henriques   +12 more
doaj   +1 more source

Erratum to: Current and Novel Aspects on the Non-lysosomal β-Glucosylceramidase GBA2 [PDF]

Neurochemical Research, 2016
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Aureli, Massimo, Samarani, Maura, Loberto, Nicoletta, Mancini, Giulia, Murdica, Valentina, Chiricozzi, Elena, Prinetti, Alessandro, Bassi, Rosaria, Sonnino, Sandro   +8 more
openaire   +2 more sources

Repression of autophagy leads to acrosome biogenesis disruption caused by a sub-chronic oral administration of polystyrene nanoparticles

Environment International, 2022
As a new widespread contaminant, nanoplastics (NPs) pose a potential risk to human health. Nevertheless, the adverse effects of NPs on the male reproductive system are poorly understood.
Lixiao Zhou, Ziying Yu, Yinyin Xia, Shuqun Cheng, Jieying Gao, Wei Sun, Xuejun Jiang, Jun Zhang, Lejiao Mao, Xia Qin, Zhen Zou, Jingfu Qiu, Chengzhi Chen   +12 more
doaj   +1 more source

Unraveling A4GALT Mechanism and Its Modulation With Adamantyl‐Galactosylceramide Analogues: Advancing Fabry Disease Therapeutic Strategies

Angewandte Chemie International Edition, EarlyView.
A 310‐helix‐mediated conformational switch promotes a front‐face SNi‐like catalysis by human A4GALT. Mechanism‐guided design identifies AdaGalCer as a selective modulator of globotriaosylceramide (Gb3) biosynthesis, opening a clear route toward new Fabry disease therapeutics.
Nicky de Koster, Òscar Vidal‐Gironès, Rowan de Graaf, Ken Kok, Lindsey T. Lelieveld, Jackelien Hoogervorst, Maria J. Ferraz, José Pablo Rivas‐Fernández, Rob F. Lammers, Herman S. Overkleeft, Johannes M. F. G. Aerts, Rolf G. Boot, Carme Rovira, Marta Artola   +13 more
wiley   +1 more source

Revealing the roles of glycosphingolipid metabolism pathway in the development of keloid: a conjoint analysis of single-cell and machine learning

Frontiers in Immunology, 2023
Keloid is a pathological scar formed by abnormal wound healing, characterized by the persistence of local inflammation and excessive collagen deposition, where the intensity of inflammation is positively correlated with the size of the scar formation ...
Binyu Song, Yu Zheng, Hao Chi, Yuhan Zhu, Zhiwei Cui, Lin Chen, Guo Chen, Botao Gao, Yichen Du, Zhou Yu, Baoqiang Song   +10 more
doaj   +1 more source

Aspartame Increases the Risk of Pancreatic Ductal Adenocarcinoma

eFood, Volume 7, Issue 3, June 2026.
Aspartame (APM) is a widely used artificial sweetener associated with various health concerns, including potential links to diabetes, cardiovascular diseases, and an increased risk of cancer. A comprehensive approach incorporating data mining, machine learning, network toxicology, molecular docking, molecular dynamics simulations, and clinical sample ...
Jumin Xie, Wei Fang, Zean Song, Zhang Yu, Ying Zhu, Shaoping Liu   +5 more
wiley   +1 more source

The Genetic Landscape of Hereditary Spastic Paraplegia in Greece

Clinical Genetics, Volume 109, Issue 5, Page 837-846, May 2026.
We investigated 112 Greek index‐cases with hereditary spastic paraplegia collected over > 25 years using NGS and MLPA. We identified a causative variant in 68 patients (60.7%), including 7 novel causative variants. This study presents a comprehensive overview of the phenotypic and genotypic spectrum of HSP in the Greek population.
Georgios Koutsis, Viorica Chelban, Chrisoula Kartanou, Zoi Kontogeorgiou, Chrysoula Koniari, Nikolaos Ragazos, Charalampos Voudommatis, Kristina Zhelcheska, David S. Lynch, Reza Maroofian, Ehsan Barkhordari, Rahema Mohammad, Ioannis Zaganas, Minas Drakos, Georgia Xiromerisiou, Efthymios Dardiotis, Leonidas Stefanis, Marios Panas, Henry Houlden, Georgia Karadima   +19 more
wiley   +1 more source

Aglycon diversity of brain sterylglucosides: structure determination of cholesteryl- and sitosterylglucoside[S]

Journal of Lipid Research, 2016
To date, sterylglucosides have been reported to be present in various fungi, plants, and animals. In bacteria, such as Helicobacter pylori, proton NMR spectral analysis of isolated 1-O-cholesteryl-β-d-glucopyranoside (GlcChol) demonstrated the presence ...
Hisako Akiyama, Kazuki Nakajima, Yoshiyuki Itoh, Tomoko Sayano, Yoko Ohashi, Yoshiki Yamaguchi, Peter Greimel, Yoshio Hirabayashi   +7 more
doaj   +1 more source