Results 41 to 50 of about 38,348 (306)

Colorectal cancer‐derived FGF19 is a metabolically active serum biomarker that exerts enteroendocrine effects on mouse liver

Molecular Oncology, EarlyView.
Meta‐transcriptome analysis identified FGF19 as a peptide enteroendocrine hormone associated with colorectal cancer prognosis. In vivo xenograft models showed release of FGF19 into the blood at levels that correlated with tumor volumes. Tumoral‐FGF19 altered murine liver metabolism through FGFR4, thereby reducing bile acid synthesis and increasing ...
Jordan M. Beardsley, Michael W. Rohr, Joseph A. Goode, Sai Preethi Nakkina, Jignesh G. Parikh, Deborah A. Altomare   +5 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

SNUPN‐Related Muscular Dystrophy: Novel Phenotypic, Pathological and Functional Protein Insights

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective SNUPN‐related muscular dystrophy or LGMDR29 is a new entity that covers from a congenital or childhood onset pure muscular dystrophy to more complex phenotypes combining neurodevelopmental features, cataracts, or spinocerebellar ataxia. So far, 12 different variants have been described.
Nuria Muelas, Pablo Iruzubieta, Alberto Damborenea, Laura Pérez‐Fernández, Inmaculada Azorín, Juan Carlos Jiménez García, Ana Töpf, Pilar Martí, Lorena Fores‐Toribio, María Manterola, Rosana Blanco‐Mañez, Oihane Pikatza‐Menoio, Sonia Alonso‐Martín, Volker Straub, Aitziber L. Cortajarena, Adolfo López de Munain, David De Sancho, Lorea Blázquez, Juan J. Vilchez   +18 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

Oral Dosed Organo‐Silica Nanoparticles Restore Glucose Homeostasis and β‐Cell Function in Diabetes Rats

Advanced Functional Materials, EarlyView.
An oral nanoplatform, MOP@T@D, which can maintain glucose homeostasis and restore islet β cells in diabetic rats is developed. It achieves efficient intestinal absorption and liver‐targeted delivery. The nanoparticle disintegrates only in response to hyperglycemia to release insulin on demand and provides antioxidant protection through selenoprotein ...
Chenxiao Chu, Mingli Wei, Che Bian, Xiaoshuang Bi, Yaxin Deng, Peifu Xiao, Jiansong Zhao, Yuying Wang, Haibing He, Jingxin Gou, Tian Yin, Xing Tang, Li Yang, Hongbo Zhang, Yu Zhang   +14 more
wiley   +1 more source

siRNA Delivery via Cross‐Linked Gelatin Microparticles Enables Targeted Modulation of Osteogenic‐Vascular Cross‐Talk: An Advanced Human 3D in Vitro Test System for Therapeutic siRNA

Advanced Healthcare Materials, EarlyView.
Osteogenic‐angiogenic cross‐talk is a vital prerequisite for vascularized bone regeneration. In this study, we investigated the effects of siRNA‐mediated silencing of two inhibitory proteins, Chordin and WWP‐1, via CaP‐NP‐loaded gelatin microparticles in osteogenically differentiated microtissues.
Franziska Mitrach, Jonas Kubat, Stefan Simm, Alexandra H. Springwald, Burak Demir, Anton Liebezeit, Michael C. Hacker, Michaela Schulz‐Siegmund   +7 more
wiley   +1 more source

High‐Throughput In Vivo Screening Using Barcoded mRNA Identifies Lipid Nanoparticles With Extrahepatic Tropism for In Situ Immunoengineering

Advanced Materials, EarlyView.
A high‐throughput in vivo mRNA LNP screening platform is developed and employed to screen a large library of 122 mRNA LNPs in vivo for delivery to immune, stromal, and parenchymal cells, identifying promising LNP candidates. A novel small particle flow cytometry‐based protein adsorption analysis method is utilized to interrogate protein corona ...
Alex G. Hamilton, Ajay S. Thatte, Junchao Xu, Zhangyi Luo, Hannah C. Safford, Kelsey L. Swingle, Jenna Muscat‐Rivera, Michael Kegel, Xuexiang Han, Ryann A. Joseph, Amanda M. Murray, Hannah C. Geisler, Ricardo C. Whitaker, Lulu Xue, Roman Spektor, Jilian R. Melamed, Drew Weissman, Michael J. Mitchell   +17 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

Liver Transplantation for Glycogen Storage Disease Type IV

Frontiers in Pediatrics, 2021
Glycogen storage disease type IV (GSD IV) is a rare autosomal recessive disorder caused by glycogen–branching enzyme (GBE) deficiency, leading to accumulation of amylopectin–like glycogen that may damage affected tissues.
Min Liu, Min Liu, Li-Ying Sun, Li-Ying Sun, Li-Ying Sun   +4 more
doaj   +1 more source

Epstein‐Barr Virus Expressed Long Non‐Coding RNA (lncBARTs) Regulate EBV Latent Genome Replication

Advanced Science, EarlyView.
EBV produces abundant level of lncBARTs, which are essential for maintaining viral genome replication in EBV‐associated cancers. LncBARTs interact with a complex comprising BRD4, CTCF and viral protein EBNA1 at EBV oriP region. This interaction tethers oriP to host chromosomes, facilitating EBV episome replication.
Jiayan Liu, Dittman Lai‐Shun Chung, Larry Ka‐Yue Chow, Shuo Han, Wenkai Yi, Conor J. Cremin, Yingyin Liao, Pui Wang, Bobo Wing‐Yee Mok, Mingfang Ji, Jian Yan, Wei Dai, Honglin Chen   +12 more
wiley   +1 more source