Results 51 to 60 of about 2,570 (187)

Exercise Intervention for REM Sleep Behavior Disorder in Parkinson's Disease: Mechanisms and Implications for Neurorehabilitation

CNS Neuroscience &Therapeutics, Volume 32, Issue 6, June 2026.
This review systematically explores exercise as a multi‐target intervention for RBD, highlighting its mechanisms of action and regulatory effects on key pathophysiological processes. ABSTRACT Backgrounds REM sleep behavior disorder (RBD) is a prodromal non‐motor symptom of Parkinson's disease (PD) and other α‐synucleinopathies, affecting approximately ...
Xinhui Qiu, Zihui Wang, Ruixue Xu, Yiying Hu, Weidong Le   +4 more
wiley   +1 more source

Molecular Basis of Reduced Glucosylceramidase Activity in the Most Common Gaucher Disease Mutant, N370S [PDF]

Journal of Biological Chemistry, 2010
Gaucher disease is caused by the defective activity of the lysosomal hydrolase, glucosylceramidase. Although the x-ray structure of wild type glucosylceramidase has been resolved, little is known about the structural features of any of the >200 mutations.
Marc N, Offman, Marcin, Krol, Israel, Silman, Joel L, Sussman, Anthony H, Futerman   +4 more
openaire   +2 more sources

Identification and Development of Biphenyl Substituted Iminosugars as Improved Dual Glucosylceramide Synthase/Neutral Glucosylceramidase Inhibitors

, 2016
This work details the evaluation of a number of N-alkylated deoxynojirimycin derivatives on their merits as dual glucosylceramide synthase/neutral glucosylceramidase inhibitors.
Wilma E. Donker-Koopman (1720321), Herman S. Overkleeft (128584), Adrianus M. C. H. van den Nieuwendijk (1720327), Tom Wennekes (1479043), Johannes M. F. G. Aerts (128594), Mark Leemhuis (1720315), Gijsbert A. van der Marel (1537651), Richard J. B. H. N. van den Berg (1720306), Arnold van Loevezijn (1720330), Anneke Strijland (1720312), Amar T. Ghisaidoobe (1720303), Saleem S. Butt (1720309), Saskia Scheij (1720318), Gerrit-Jan Koomen (1720324), Mario van der Stelt (222751), Constant A. A. van Boeckel (120036)   +15 more
core   +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

Acid ceramidase overactivity drives ceramide loss, leading to atopic dry skin and Th2‐skewed immune polarization

The Journal of Pathology, Volume 269, Issue 2, Page 232-247, June 2026.
Abstract Ceramide deficiency in the stratum corneum (SC) is a key etiological factor in atopic dermatitis (AD). To clarify the direct role of SC ceramide depletion in impairing SC barrier and water‐holding functions and in initiating AD‐like skin symptoms and disease‐specific molecular alterations, we generated Tg mice overexpressing a mutant form of ...
Mariko Takada, Miho Sashikawa‐Kimura, Yusuke Ohno, Md Razib Hossain, Xiaonan Xie, Kazuhisa Iwabuchi, Mayumi Komine, Mamitaro Ohtsuki, Genji Imokawa   +8 more
wiley   +1 more source

Distinguishing the differences in β-glycosylceramidase folds, dynamics, and actions informs therapeutic uses

Journal of Lipid Research, 2018
Glycosyl hydrolases (GHs) are carbohydrate-active enzymes that hydrolyze a specific β-glycosidic bond in glycoconjugate substrates; β-glucosidases degrade glucosylceramide, a ubiquitous glycosphingolipid.
Fredj Ben Bdira, Marta Artola, Herman S. Overkleeft, Marcellus Ubbink, Johannes M.F.G. Aerts   +4 more
doaj   +1 more source

Glucocerebrosidase deficiency promotes protein aggregation through dysregulation of extracellular vesicles. [PDF]

PLoS Genetics, 2018
Mutations in the glucosylceramidase beta (GBA) gene are strongly associated with neurodegenerative diseases marked by protein aggregation. GBA encodes the lysosomal enzyme glucocerebrosidase, which breaks down glucosylceramide.
Ruth E Thomas, Evelyn S Vincow, Gennifer E Merrihew, Michael J MacCoss, Marie Y Davis, Leo J Pallanck   +5 more
doaj   +1 more source

Autosomal recessive cerebellar ataxia with spasticity due to a rare mutation in GBA2 gene in a large consanguineous Saudi family

Genes and Diseases, 2021
The nonlysosomal glucosylceramidase β2 (GBA2) gene encode an enzyme that catalyzes the hydrolysis of glucosylceramide to glucose and ceramide. Mutations in the GBA2 gene have been reported to cause hereditary spastic paraplegia, autosomal recessive ...
Hussein Algahtani, Bader Shirah, Ikram Ullah, Mohammad H. Al-Qahtani, Angham Abdulrahman Abdulkareem, Muhammad Imran Naseer   +5 more
doaj   +1 more source

Preclinical investigation of artesunate as a therapeutic agent for hepatocellular carcinoma via impairment of glucosylceramidase-mediated autophagic degradation

Experimental & Molecular Medicine, 2022
AbstractArtesunate (ART) has been indicated as a candidate drug for hepatocellular carcinoma (HCC). Glucosylceramidase (GBA) is required for autophagic degradation. Whether ART regulates autophagic flux by targeting GBA in HCC remains to be defined. Herein, our data demonstrated that the dramatic overexpression of GBA was significantly associated with ...
Wenjia Chen, Zhaochen Ma, Lingxiang Yu, Xia MAO, Nan Ma, Xiaodong Guo, Xiaoli Yin, Funeng Jiang, Qian Wang, Jigang Wang, Mingliang Fang, Na Lin, Yanqiong Zhang   +12 more
openaire   +2 more sources

Reducing GBA2 Activity Ameliorates Neuropathology in Niemann-Pick Type C Mice.

PLoS ONE, 2015
The enzyme glucocerebrosidase (GBA) hydrolyses glucosylceramide (GlcCer) in lysosomes. Markedly reduced GBA activity is associated with severe manifestations of Gaucher disease including neurological involvement.
André R A Marques, Jan Aten, Roelof Ottenhoff, Cindy P A A van Roomen, Daniela Herrera Moro, Nike Claessen, María Fernanda Vinueza Veloz, Kuikui Zhou, Zhanmin Lin, Mina Mirzaian, Rolf G Boot, Chris I De Zeeuw, Herman S Overkleeft, Yildiz Yildiz, Johannes M F G Aerts   +14 more
doaj   +1 more source