Results 31 to 40 of about 36,696 (264)

MASH Background Confers Enhanced Disease Susceptibility and Acetaminophen Toxicity in iPSC‐Derived Liver Organoids

Advanced Science, EarlyView.
This work establishes a novel method for generating multicellular liver organoids from control and MASH donor iPSCs. The model recapitulates several disease‐specific characteristics, with MASH donor‐derived organoids showing higher susceptibility. Lipidomic profiling of MASH organoids closely resembles MASH liver biopsies.
Ekta Minocha, Ashwani Kumar Gupta, Nate Schmidt, Richard M. Green, John G. Purdy, Jason A. Wertheim   +5 more
wiley   +1 more source

Current Clinical Guidelines for the Management of Patients with Glycogen Storage Disease

Педиатрическая фармакология
Glycogen storage disease refers to hereditary pathologies of carbohydrate metabolism, its cause is mutations of various genes encoding enzymes responsible for the synthesis and breakdown of glycogen.
Natalia A. Averkina, Madlena E. Bagaeva, Aleksander A. Baranov, Nato D. Vashakmadze, Elena A. Vishneva, Olga S. Gundobina, Nataliya V. Zhurkova, Elena V. Kaitukova, Elena V. Komarova, Tea V. Margieva, Leyla S. Namazova-Baranova, Valeria P. Novikova, Elena E. Petryaykina, Mariya M. Platonova, Aleksander S. Potapov, Olga Ya. Smirnova, Tatiana V. Strokova, Andrey N. Surkov, Nataliya N. Taran, Marina V. Fedoseenko, Nataliya A. Semenova, Inga V. Anisimova, Svetlana A. Repina, Dmitriy M. Subbotin, Valeria V. Sviridova, Anatoliy I. Havkin, Ekaterina A. Yablokova, Galina V. Volynets, Irina V. Sadovnikova, Elena L. Tumanova   +29 more
doaj   +1 more source

Optimized Ex Vivo Human Liver Slice Culture Maintains Extended Viability and Function for Hepatotoxicity Testing

Advanced Science, EarlyView.
An optimized ex vivo human liver slice culture system preserves tissue architecture, hepatocyte function, and immune‐stromal complexity for at least five days. By refining slice thickness, oxygenation, air‐liquid interface, and extracellular matrix support, the platform enables human‐relevant hepatotoxicity testing and mechanistic studies, offering a ...
Huiche Feng, Cinzia Esposito, Andrej Benjak, Matthias S. Matter, Miriam Cieri, Camilla De Carlo, Philipp Sedlaczek, Fabian Haak, Mattia Marinucci, Gabriel Fridolin Hess, Otto Kollmar, Mairene Coto‐Llerena, Luca Di Tommaso, Visar Vela, Lukas Bubendorf, Luigi M. Terracciano, Charlotte K. Y. Ng, Salvatore Piscuoglio   +17 more
wiley   +1 more source

Multimodal Imaging Reveals Rapid Catecholamine Uptake and Release by Neutrophils

Advanced Science, EarlyView.
We show that immune cells (neutrophils) synthesize, uptake, and store catecholamine neurotransmitters such as dopamine or adrenaline. They also release them in response to specific stimuli (serotonin), which we directly visualize using fluorescent nanosensors. We further demonstrate that catecholamines affect neutrophil functions (NETosis) and platelet
Jennifer Mohr, Anne Schmitz, Meshkat Dinarvand, Franziska Wulfert, Sangeetha Shankar, Bjoern F. Hill, Michael Wojak, Juliana Gretz, Marie Britz, Elsa Neubert, Magdalena Shumanska, Sofia Kaushik, Linda Kartaschew, Ivan Bogeski, James Daniel, Sebastian Jung, Johannes Eble, Guido Wabnitz, Luise Erpenbeck, Sebastian Kruss   +19 more
wiley   +1 more source

A Brain‐Penetrant Nanobody Reveals GSK3β‐Driven Proline‐Directed Phosphorylation as a Master Regulator of Ischemic Neurodegeneration

Advanced Science, EarlyView.
A brain‐targeted nanoparticle enables delivery of a therapeutic nanobody (Nb.29E9) that inhibits pathogenic GSK3β signaling. This intervention restores AMPK/mTORC1/TGFβ homeostasis, attenuates neuroinflammation and oxidative stress, and promotes long‐term functional recovery after ischemic stroke.
Lan Li, Muyang Li, Lei Sun, Ying Yang, Yuanshun Wu, Ziyi Yin, Anni Wang, Peiyang Zhou, Shaoxiang Luo, Jian Chen, Jun Qin, Zhibing Ai, Zilong Yuan, Zhiqiang Dong, Min Zhang   +14 more
wiley   +1 more source

Report of an Iranian child with chronic abdominal pain and constipation diagnosed as glycogen storage disease type IX: a case report

Journal of Medical Case Reports
Background Glycogen storage disease type IX is a rare disorder that can cause a wide variety of symptoms depending on the specific deficiency of the phosphorylase kinase enzyme and the organs it affects.
Daniel Zamanfar, Seyed MohammadBagher Hashemi-Soteh, Mobin Ghazaiean, Elham Keyhanian   +3 more
doaj   +1 more source

Expanding the clinicopathological-genetic spectrum of glycogen storage disease type IXd by a Chinese neuromuscular center

Frontiers in Neurology, 2022
BackgroundGlycogen storage disease (GSDs) is characterized by abnormally inherited glycogen metabolism. GSD IXd, which is caused by mutations in the PHKA1 gene, is an X-linked rare disease with mild myopathic symptoms.
Kun Huang, Kun Huang, Hui-Qian Duan, Hui-Qian Duan, Qiu-Xiang Li, Qiu-Xiang Li, Yue-Bei Luo, Yue-Bei Luo, Fang-Fang Bi, Fang-Fang Bi, Huan Yang, Huan Yang   +11 more
doaj   +1 more source

A Single‐Cell Transcriptomic Atlas of the Ovine Rumen Microbiome Characterizes Lineage‐Specific Metabolic Shifts Associated with Host Heat Tolerance

Advanced Science, EarlyView.
An optimized single‐cell transcriptomic framework profiles over 60 000 cells to map the ovine rumen microbiome, partitioning the ecosystem into seven cross‐species functional clusters. In heat‐resistant hosts, a lineage‐specific metabolic shift in Anaerovibrio lipolyticus toward a highly glycolytic phenotype contributes to a “nutritional sparing ...
Sanbao Zhang, Qinyang Jiang, Junjie Ma, Jianwei Zou, Fan Wang, Feifei Lv, Yanna Huang, Yongcheng Wang, Ziye Xu   +8 more
wiley   +1 more source

Vascularized Liver‐on‐a‐Microsphere Reveals Alanine‐Glucose Metabolism‐Driven Regulation of Liver Function and Injury

Advanced Science, EarlyView.
A vascularized liver‐on‐a‐microsphere system is developed to recreate lobule‐like cellular organization and endothelial barrier function within compartmentalized hydrogel particles. The platform enhances hepatic performance and reveals alanine‐glucose cycle activation linked to improved detoxification capacity, offering a high‐throughput microscale ...
Jingyang Li, Zengnan Wu, Yingrui Zhang, Shulang Chen, Yongning Lin, Shiyu Chen, Tong Xu, Xianli Meng, Yi Zhang, Jin‐Ming Lin   +9 more
wiley   +1 more source

Alleviation of a polyglucosan storage disorder by enhancement of autophagic glycogen catabolism

EMBO Molecular Medicine, 2021
This work employs adult polyglucosan body disease (APBD) models to explore the efficacy and mechanism of action of the polyglucosan‐reducing compound 144DG11.
Or Kakhlon, Hilla Vaknin, Kumudesh Mishra, Jeevitha D’Souza, Monzer Marisat, Uri Sprecher, Shane Wald‐Altman, Anna Dukhovny, Yuval Raviv, Benny Da’adoosh, Hamutal Engel, Sandrine Benhamron, Keren Nitzan, Sahar Sweetat, Anna Permyakova, Anat Mordechai, Hasan Orhan Akman, Hanna Rosenmann, Alexander Lossos, Joseph Tam, Berge A. Minassian, Miguel Weil   +21 more
doaj   +1 more source