Results 61 to 70 of about 7,225 (221)

Intestinal Neutral Ceramidase Deficiency Triggers Regulatory T Cell Response via Gd3 to Protect the Host from Intestinal Inflammation

Advanced Science, Volume 13, Issue 5, 27 January 2026.
Deletion of ASAH2 in intestinal epithelial cells induces the accumulation of ganglioside GD3 via regulating ST8SIA1. GD3 is identified as the glycolipid ligand for the inhibitory receptor Siglec‐E on macrophages. GD3/Siglec‐E ligation polarizes macrophages and promotes the proliferation of colonic ST2+ T regulatory cells via the induction of IL‐33. The
Zhishan Xu, Chao Lei, Mukesh K Sriwastva, Ting Wang, Amanguli Tuohongerbieke, Xiaotong Song, Collin Noud, Shannon Derkson, Yi Tan, Gerald Dryden, Craig J McClain, Zhongbin Deng   +11 more
wiley   +1 more source

Contribution of shape and charge to the inhibition of a family GH99 endo-α-1,2-mannanase [PDF]

, 2017
[Image: see text] Inhibitor design incorporating features of the reaction coordinate and transition-state structure has emerged as a powerful approach for the development of enzyme inhibitors.
Davies, Gideon John, Fernandes, Pearl Z., Ganeko, Bernardo-Seisdedos, Jiménez-Barbero, Jesús, Millet, Oscar, Petricevic, Marija, Raich, Lluís, Rovira, Carme, Sobala, Lukasz, Sollogoub, Matthieu, Thompson, Andrew James, Williams, Spencer J., Zhu, Sha   +12 more
core   +4 more sources

Stromal Cell‐Mast Cell Communication Orchestrates Anti‐Viral Immunity in the Meninges

Advanced Science, Volume 13, Issue 5, 27 January 2026.
This study delineates the spatial distribution and postnatal maturation of meningeal mast cells and uncovers their essential role in antiviral immunity. Using genetic models, mast cells are shown to promote CD8⁺ T cell infiltration and viral clearance via IL‐33 receptor signaling and synergistic activation by IL‐33 and ATP from infected stromal cells ...
Qingqing Li, Weijia Chen, Mengxue Sun, Xinbo Ni, Qishan Ran, Xiaoyu Hu, Fang Cao, Wenwen Zeng   +7 more
wiley   +1 more source

First Reported Case of CLN5 Disease in Japan: Identification of a Novel Homozygous Pathogenic Variant Through Whole Genome Sequencing

Clinical Case Reports, Volume 14, Issue 1, January 2026.
ABSTRACT Neuronal ceroid lipofuscinoses (NCL) belong to a group of inherited neurodegenerative diseases characterized by psychomotor regression, seizures, and visual impairment, resulting from intracellular accumulation of lipofuscin. CLN5, a subtype typically manifesting between ages 4 to 17, is particularly rare in non‐Finnish populations.
Eriko Nishi, Kumiko Yanagi, Morimasa Shima, Naoko Yamazaki, Kazumi Kawato, Aya Narita, Norio Sakai, Nobuhiko Okamoto, Keiko Yanagihara, Tadashi Kaname   +9 more
wiley   +1 more source

Isoenzymes of N-acetyl-beta-hexosaminidase.

Acta Biochimica Polonica, 1999
Biological significance, structure and posttranslational processing of N-acetyl-beta-hexosaminidase isoenzymes are described. Clinical application of N-acetyl-beta-hexosaminidase is also reviewed.
K, Zwierz, A, Zalewska, A, Zoch-Zwierz
openaire   +3 more sources

Exploring the Role of Acrylamide in Food Allergy via Dendritic Cell‐Dependent Th2 Immune Responses

Food Frontiers, Volume 7, Issue 1, January 2026.
Acrylamide may disrupt immune tolerance as a foodborne immunotoxicant, priming allergic inflammatory susceptibility through DC‐dependent Th2/Th17 polarization, TLR4/NF‐κB signaling amplification, and IL‐10 production suppression. ABSTRACT Acrylamide (AA), a prevalent food processing contaminant with recognized multi‐organ toxicity, has an unexplored ...
Xue Feng, Jiamin Chen, Zhencong Yang, Philippe Madjirebaye, Zhoujin Xu, Li Yan, Yuting Fan, Xiaoliang Shu, Xuli Wu   +8 more
wiley   +1 more source

Phylogenetic analyses suggest multiple changes of substrate specificity within the Glycosyl hydrolase 20 family [PDF]

, 2008
Background Beta-N-acetylhexosaminidases belonging to the glycosyl hydrolase 20 (GH20) family are involved in the removal of terminal β-glycosidacally linked N-acetylhexosamine residues.
Horner, David S, Intra, Jari, Pavesi, Giulio   +2 more
core   +4 more sources

Therapeutic Effects of Nizubaglustat in a Mouse Model of GM2 Gangliosidosis

Journal of Inherited Metabolic Disease, Volume 49, Issue 1, January 2026.
ABSTRACT Nizubaglustat is a novel selective inhibitor of glucosylceramide synthase (GCS) and the non‐lysosomal glucocerebrosidase (NLGase, GbA2) with brain penetrant properties. It is currently in clinical development as an oral treatment for rare lysosomal storage diseases with neurological involvement. One such disease group called GM2 gangliosidosis,
Kyle Landskroner, Kshitiz Singh, Melissa Mitchell, Jagdeep S. Walia   +3 more
wiley   +1 more source

Stereoselective synthesis of 2-acetamido-1,2-dideoxynojirimycin (DNJNAc) and ureido-DNJNAc derivatives as new hexosaminidase inhibitors [PDF]

, 2015
2-Acetamido-1,2-dideoxyiminosugars are selective and potent inhibitors of hexosaminidases and therefore show high therapeutic potential for the treatment of various diseases, including several lysosomal storage disorders. A stereoselective synthesis of 2-
Farrar-Tobar, Ronald A., Fuente, Alex de la, García Fernández, José Manuel, Mena Barragán, Teresa, Ortiz Mellet, Carmen, Riera, Antoni, Verdaguer, Xavier   +6 more
core   +3 more sources

The physiological and pathological effects of sphingolipid metabolism and signaling in the central nervous system

Brain Pathology, Volume 36, Issue 1, January 2026.
Sphingolipids are vital components of cell membranes. Metabolic disruptions of sphingolipids, including ceramide and sphingosine‐1‐phosphate, are linked to neurological disorders. This article summarizes the classification, structure, and metabolic processes of sphingolipids, and the physiological and pathological effects of sphingolipid metabolism and
Tian Li, Haoying He, Ejuan Zhang, Fengjiao Hu, Zhuo Wang, Jian Xu, Mengliu Zeng, Biwen Peng   +7 more
wiley   +1 more source