Results 41 to 50 of about 25,377 (250)

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

Glycogen storage disease type I (GSD I) [PDF]

Atlas of Genetics and Cytogenetics in Oncology and Haematology, 2012
Review on Glycogen storage disease type I (GSD I), with data on clinics, and the genes involved.
Mollet, Boudjemline A, Petit, F, Hubert, Buron A, Trioche, Eberschweiler P, Gajdos, V, Labrune, P   +5 more
openaire   +2 more sources

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

Newborn Screening for Pompe Disease

International Journal of Neonatal Screening, 2020
Glycogen storage disease type II (also known as Pompe disease (PD)) is an autosomal recessive disorder caused by defects in α-glucosidase (AαGlu), resulting in lysosomal glycogen accumulation in skeletal and heart muscles.
Takaaki Sawada, Jun Kido, Kimitoshi Nakamura   +2 more
doaj   +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

Glucose-6 Phosphate, A Central Hub for Liver Carbohydrate Metabolism

Metabolites, 2019
: Cells efficiently adjust their metabolism according to the abundance of nutrients and energy. The ability to switch cellular metabolism between anabolic and catabolic processes is critical for cell growth.
Fabienne Rajas, Amandine Gautier-Stein, Gilles Mithieux   +2 more
doaj   +1 more source

Liver transplantation for type I and type IV glycogen storage disease [PDF]

European Journal of Pediatrics, 1993
Progressive liver failure or hepatic complications of the primary disease led to orthotopic liver transplantation in eight children with glycogen storage disease over a 9-year period. One patient had glycogen storage disease (GSD) type I (von Gierke disease) and seven patients had type IV GSD (Andersen disease). As previously reported [19], a 16.5-year-
Selby, R, Starzl, TE, Yunis, E, Todo, S, Tzakis, AG, Brown, BI, Kendall, RS   +6 more
openaire   +2 more sources

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

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