Article
Multidisciplinary Sciences
Sumate Ampawong, Napatara Tirawanchai, Tapanee Kanjanapruthipong, Kamonpan Fongsodsri, Khwanchanok Tuentam, Duangnate Isarangkul, Pornanong Aramwit
Summary: This study aims to examine the effect of sericin on urea cycle enzyme genes and proteins, and found that sericin can promote ammonia detoxification by increasing urea cycle enzyme genes and enhancing hepatic autophagy.
Article
Neurosciences
Ruimiao Bai, ALing He, Jinzhen Guo, Zhankui Li, Xiping Yu, JunAn Zeng, Yang Mi, Lin Wang, Jingjing Zhang, Dong Yang
Summary: In this study, two pathogenic missense variants of carbamoyl phosphate synthetase 1 deficiency (CPS1D) were identified using next-generation sequencing (NGS) in an Asian neonatal patient. The variants were predicted to be deleterious based on various bioinformatical analyses. This study expands the variant spectrum of the CPS1 gene and demonstrates the applicability of variants and related structural knowledge in the accurate diagnosis of CPS1D.
FRONTIERS IN NEUROSCIENCE
(2022)
Article
Medical Laboratory Technology
Yunha Choi, Arum Oh, Yena Lee, Gu-Hwan Kim, Jin-Ho Choi, Han-Wook Yoo, Beom Hee Lee
Summary: CPS1 deficiency presents with the most severe clinical manifestations among urea cycle disorders, with a high proportion of missense mutations, and late-onset patients showing dramatic response to N-carbamylglutamate treatment.
CLINICA CHIMICA ACTA
(2022)
Article
Microbiology
Xunhui Zhuo, Kaige Du, Haojie Ding, Di Lou, Bin Zheng, Shaohong Lu
Summary: The study showed that a mutant with complete deletion of CPSII exhibited significantly reduced replication in vitro and decreased virulence in mice, leading to enhanced survival rates. This suggests that strains lacking the de novo pyrimidine biosynthesis pathway and salvage pathway may have potential as a live attenuated vaccine to prevent T. gondii infection.
FRONTIERS IN MICROBIOLOGY
(2021)
Article
Virology
Ze Xiang, Bin Jiang, Wei Li, Guanghua Zhai, Hao Zhou, Ying Wang, Jian Wu
Summary: This study found that serum exosome-derived CPS1 levels were significantly elevated in HEV-ALF patients and were closely associated with organ failure and prognosis. Serum exosome-derived CPS1 levels may serve as a promising diagnostic and prognostic biomarker for HEV-ALF patients.
JOURNAL OF MEDICAL VIROLOGY
(2022)
Article
Pediatrics
Nashwa El-Khazragy, Mohamed El Barbary, Hala Fouad, Abdallah Abdelgawad, Dina Rabie
Summary: The study found a significant association between CPS-I gene polymorphism and idiopathic PPHN in Egyptian neonates, especially the CPS-I A/C rs4399666 gene variant and lower serum NO levels. The presence of mutant CPS-I rs4399666, specifically the homozygous CC genotype, is frequently observed in the PPHN group and associated with a higher risk of PPHN.
EUROPEAN JOURNAL OF PEDIATRICS
(2021)
Article
Biochemistry & Molecular Biology
Lan Zhang, Yuling Zou, Yingying Lu, Zhijia Li, Feng Gao
Summary: CPS1 is the rate-limiting enzyme controlling the first reaction of the urea cycle, which is essential for converting toxic ammonia into non-toxic urea in mammals. Dysfunctions of CPS1 can lead to urea cycle disorder and nerve damage. Furthermore, CPS1 has been found to be involved in various human diseases, including cancers, making it a potential therapeutic target. However, the effects of small-molecule inhibitors of CPS1 in malignancies are yet to be experimentally confirmed.
BIOORGANIC CHEMISTRY
(2023)
Article
Cell Biology
Seung-Hee Lee, Vimal Veeriah, Fred Levine
Summary: This study reports the discovery of potent HNF4 alpha agonists and reveals a novel pathway by which HNF4 alpha controls fat storage in the liver. In vitro experiments demonstrated that the activators NCT and NFT can induce fat clearance, while in DIO mice, NCT led to recovery of hepatic HNF4 alpha expression and reduction of steatosis, suggesting NCT as a potential therapeutic for NAFLD.
CELL DEATH & DISEASE
(2021)
Article
Biochemistry & Molecular Biology
Ze-Hao Shang, Mei Huang, Meng-Xiao Wu, Di Mi, Kun You, Yun-Long Zhang
Summary: This study conducted transcriptomic analyses on the gill and liver of large-scale loach exposed to air and ammonia for 48 hours, revealing the impact of ammonia nitrogen accumulation on the species. The findings suggest that large-scale loach responds to ammonia stress by enhancing amino acid metabolism and regulating the expression of ammonia transporters in the gill.
COMPARATIVE BIOCHEMISTRY AND PHYSIOLOGY C-TOXICOLOGY & PHARMACOLOGY
(2021)
Review
Cell Biology
Yan Jiao, Ping Xu, Honglin Shi, Dexi Chen, Hongbo Shi
Summary: Exosomes are extracellular vesicles ranging in size from 30 to 150 nm, containing various proteins, mRNA, miRNA, and lipids which coordinate physiological and pathological functions through cell-to-cell communication. Specifically, liver cell-derived exosomes play a crucial role in both general physiological functions and the pathogenesis of liver diseases, making them potential diagnostic biomarkers and treatment targets.
JOURNAL OF CELLULAR AND MOLECULAR MEDICINE
(2021)
Article
Fisheries
Muzi Zhang, Shidong Wang, Liying Sun, Lei Gan, Yanhong Lin, Jian Shao, Haibo Jiang, Ming Li
Summary: Fishes can adapt to environmental ammonia, and the study found that the CPS I mRNA was significantly up-regulated in liver and kidney tissues after acute ammonia stress. The RNA interference of CPS I led to changes in enzyme activities related to urea cycle and glutamine synthesis, suggesting a shift in ammonia detoxification strategies.
FISH & SHELLFISH IMMUNOLOGY
(2022)
Article
Multidisciplinary Sciences
Louis M. P. Ter-Ovanessian, Baptiste Rigaud, Alberto Mezzetti, Jean-Francois Lambert, Marie-Christine Maurel
Summary: The study focuses on the first step of pyrimidine synthesis to test the hypothesis of geochemical continuity of protometabolic pathways at the origins of life. It found that carbamoyl phosphate is initially stable in water before transforming to cyanate and carbamate/hydrogenocarbonate species, with faster decomposition and urea generation in the presence of ammonia. The research suggests that CP may not be a likely prebiotic reagent, and cyanate and urea could be promising substitutes due to their energy-rich properties and kinetic inertness towards hydrolysis.
SCIENTIFIC REPORTS
(2021)
Article
Toxicology
Evelyn Silva Moreira, Ana Paula Ames-Sibin, Carla Indianara Bonetti, Luana Eloisa Leal, Rosane Marina Peralta, Anacharis Babeto de Sa-Nakanishi, Jurandir Fernando Comar, Adelar Bracht, Livia Bracht
Summary: Berberine, a plant alkaloid, has been recognized for its antihyperglycemic properties and inhibitory effects on mitochondrial functions. This study investigated its short-term effects on hepatic metabolism using isolated perfused rat livers. The results showed that berberine modified multiple metabolic pathways, inhibiting gluconeogenesis, increasing glycolysis, impairing ammonia detoxification, and affecting energy metabolism. These findings suggest that the direct inhibitory effects of berberine on gluconeogenesis contribute significantly to its clinical efficacy as an antihyperglycemic agent. However, safety concerns arise due to the narrow margin between expected benefits and toxicity.
TOXICOLOGY LETTERS
(2022)
Article
Medicine, General & Internal
Khaa Hoo Ong, Yao-Yu Hsieh, Ding-Ping Sun, Steven Kuan-Hua Huang, Yu-Feng Tian, Chia-Ling Chou, Yow-Ling Shiue, Keva Joseph, I-Wei Chang
Summary: Intrahepatic cholangiocarcinoma (IHCC) is more common in Eastern and Southeastern Asia than in Western countries. Through gene expression analysis, it has been found that CPS1 gene is significantly down-regulated in IHCC. The low expression of CPS1 in IHCC is associated with tumor progression and predicts poor clinical outcomes for patients.
Article
Medicine, Research & Experimental
Cuiting Hao, Xi Guo, Zhenghan Dong, Qiong Guo, Wei Shi
Summary: Malignant tumors pose an urgent global threat to human health. Compounds extracted from grains have been found to exhibit antioxidant, antiviral, and antitumor activities. By stimulating liver tumor cells with zymolytic grain extract (ZGE), the expression levels of specific genes were altered, leading to the acquisition of hepatocyte-like characteristics by the transformed cells. This discovery not only provides new insights into liver tumor treatment but also lays the groundwork for combination therapy.
BIOMEDICINE & PHARMACOTHERAPY
(2023)
