Results 21 to 30 of about 3,985 (137)

Underlying mechanisms of phosphodiesterase 10A and glutamate-ammonia ligase genes that regulate inosine monophosphate deposition and thereby affect muscle tenderness in Jingyuan chickens [PDF]

Animal Bioscience, 2022
Objective Inosine monophosphate (IMP) is a key factor that imparts of meat flavor. Differences in the IMP content in the muscles were evaluated to improve chicken meat quality.
Weizhen Wang, Juan Zhang, Honghong Hu, Baojun Yu, Jintong He, Tingting Yao, Yaling Gu, Zhengyun Cai, Guosheng Xin   +8 more
doaj   +1 more source

Homozygous GLUL deletion is embryonically viable and leads to glutamine synthetase deficiency [PDF]

Clinical Genetics, 2020
AbstractGlutamine synthetase (GS) is the enzyme responsible for the biosynthesis of glutamine, providing the only source of endogenous glutamine necessary for several critical metabolic and developmental pathways. GS deficiency, caused by pathogenic variants in the glutamate‐ammonia ligase (GLUL) gene, is a rare autosomal recessive inborn error of ...
Roifman, Maian, Niles, Kirsten M, MacNeil, Lauren, Blaser, Susan, Noor, Abdul, Godoy, Ruth, van Mieghem, Tim, Ryan, Greg, Seaward, Gareth, Sondheimer, Neal, Mercimek-Andrews, Saadet, Schulze, Andreas, Hewson, Stacy, Ovadia, Adi, Chitayat, David, Morgen, Eric K, Hojilla, Carlo, Kolomietz, Elena, Watkins, Nicholas, Häberle, Johannes, Shannon, Patrick   +20 more
openaire   +3 more sources

FISH mapping of three ammonia metabolism genes (Glul, Cps1, Glud1) in rat, and the chromosomal localization of GLUL in human and Cps1 in mouse [PDF]

Mammalian Genome, 1997
In recent years, the rat genes encoding glutamate dehydrogenase (GLUD; Das et al. 1993), glutamine synthetase (glutamateammonia ligase, GLUL; van de Zande et al. 1990) and carbamoylphosphate synthetase 1 (CPS; Van den Hoff et al. 1995) have been isolated.
Helou, Khalil, Das, A T, Lamers, W H, Hoovers, J M, Szpirer, Claude, Szpirer, Josiane, Klinga-Levan, K, Levan, G   +7 more
openaire   +4 more sources

PHF8-GLUL axis in lipid deposition and tumor growth of clear cell renal cell carcinoma

Science Advances, 2023
For clear cell renal cell carcinoma (ccRCC), lipid deposition plays important roles in the development, metastasis, and drug resistance. However, the molecular mechanisms underlying lipid deposition in ccRCC remain largely unknown. By conducting an unbiased CRISPR-Cas9 screening, we identified the epigenetic regulator plant homeodomain ...
Song Peng, Ze Wang, Peng Tang, Shuo Wang, Yiqiang Huang, Qiubo Xie, Yapeng Wang, Xintao Tan, Tang Tang, Xuzhi Yan, Jing Xu, Weihua Lan, Luofu Wang, Dianzheng Zhang, Bin Wang, Tiejun Pan, Jun Qin, Jun Jiang, Qiuli Liu   +18 more
openaire   +2 more sources

Multiomics identifies the link between intratumor steatosis and the exhausted tumor immune microenvironment in hepatocellular carcinoma

Hepatology, EarlyView., 2022
Steatotic HCC can be diagnosed by MRI and forms a pro‐tumor immune microenvironment with high expression of PD‐L1, M2 polarization of macrophages and activation of CAFs, which may be a good target for combined immunotherapy. Abstract Background and Aims Immunotherapy has become the standard‐of‐care treatment for hepatocellular carcinoma (HCC), but its ...
Hiroki Murai, Takahiro Kodama, Kazuki Maesaka, Shoichiro Tange, Daisuke Motooka, Yutaka Suzuki, Yasuyuki Shigematsu, Kentaro Inamura, Yoshihiro Mise, Akio Saiura, Yoshihiro Ono, Yu Takahashi, Yota Kawasaki, Satoshi Iino, Shogo Kobayashi, Masashi Idogawa, Takashi Tokino, Tomomi Hashidate‐Yoshida, Hideo Shindou, Masanori Miyazaki, Yasuharu Imai, Satoshi Tanaka, Eiji Mita, Kazuyoshi Ohkawa, Hayato Hikita, Ryotaro Sakamori, Tomohide Tatsumi, Hidetoshi Eguchi, Eiichi Morii, Tetsuo Takehara   +29 more
wiley   +1 more source

ALK1 controls hepatic vessel formation, angiodiversity, and angiocrine functions in hereditary hemorrhagic telangiectasia of the liver

Hepatology, EarlyView., 2022
Hepatic endothelial Alk1 signaling protects from development of vascular malformations while maintaining organ‐specific endothelial differentiation and angiocrine portmanteau of the names Wingless and Int‐1 signaling. Abstract Background and Aims In hereditary hemorrhagic telangiectasia (HHT), severe liver vascular malformations are associated with ...
Christian David Schmid, Victor Olsavszky, Manuel Reinhart, Vanessa Weyer, Felix A. Trogisch, Carsten Sticht, Manuel Winkler, Sina W. Kürschner, Johannes Hoffmann, Roxana Ola, Theresa Staniczek, Joerg Heineke, Beate K. Straub, Jens Mittler, Kai Schledzewski, Peter ten Dijke, Karsten Richter, Steven Dooley, Cyrill Géraud, Sergij Goerdt, Philipp‐Sebastian Koch   +20 more
wiley   +1 more source

GLUL mediates FOXO3 O-GlcNAcylation to regulate the osteogenic differentiation of BMSCs and senile osteoporosis. [PDF]

Cell Death Differ
Abstract The abnormal osteogenic differentiation of bone marrow mesenchymal stem cells (BMSCs) is an important cause of senile osteoporosis (SOP). Glutamine synthetase (GLUL) is a key enzyme in glutamine biosynthesis; however, its functional role in SOP remains unclear. Here, we found that GLUL expression was downregulated in the BMSCs of SOP
Zhang L, Qi B, Li Y, Liang X, Zhang Z, Yang T, Jia S, Gao X, Chen S, Jiao G, Li Y, Zhou H, Chen Y, Li Y, Zhang B, Li G, Meng C.   +16 more
europepmc   +2 more sources

Two Siblings With Valproate-Related Hyperammonemia and Novel Mutations in Glutamine Synthetase (GLUL) Treated With Carglumic Acid [PDF]

Child Neurology Open, 2020
This case report describes 2 siblings with myoclonic epilepsy who had novel mutations in the glutamine synthetase ( GLUL) gene: c.316C>T, p.(Arg106*) and c.42G>C, p.(Lys14Asn). Valproic acid improved seizure control, but was associated with hyperammonemic encephalopathy.
Jennifer Bennett, Christy Gilkes, Karin Klassen, Marina Kerr, Aneal Khan   +4 more
openaire   +2 more sources

T Helper Cell Activation and Expansion Is Sensitive to Glutaminase Inhibition under Both Hypoxic and Normoxic Conditions. [PDF]

PLoS ONE, 2016
Immune responses often take place where nutrients and O2 availability are limited. This has an impact on T cell metabolism and influences activation and effector functions.
Zeynep Sener, Fritjof H Cederkvist, Roman Volchenkov, Halvor L Holen, Bjørn S Skålhegg   +4 more
doaj   +1 more source

Increased NMDARs in neurons and glutamine synthetase in astrocytes underlying autistic-like behaviors of Gabrb1−/− mice

iScience, 2023
Summary: Mutations of the GABA-A receptor subunit β1 (GABRB1) gene are found in autism patients. However, it remains unclear how mutations in Gabrb1 may lead to autism. We generated Gabrb1−/− mouse model, which showed autistic-like behaviors.
Jing Wang, Yue Gao, Liuyan Xiao, Yanmei Lin, Lang Huang, Jinfa Chen, Guanmei Liang, Weiming Li, Wenjuan Yi, Jianpei Lao, Bin Zhang, Tian-Ming Gao, Mei Zhong, Xinping Yang   +13 more
doaj   +1 more source