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Carbamoyl-phosphate synthase 1, mitochondrial

Carbamoyl-Phosphate Synthase
The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.[provided by RefSeq, May 2010] (from NCBI)
Top mentioned proteins: ACID, HAD, Aspartate Carbamoyltransferase, AGE, CAN
Papers on Carbamoyl-Phosphate Synthase
Diversion of aspartate in ASS1-deficient tumours fosters de novo pyrimidine synthesis.
Erez et al., Israel. In Nature, Dec 2015
Here we show that decreased activity of ASS1 in cancers supports proliferation by facilitating pyrimidine synthesis via CAD (carbamoyl-phosphate synthase 2, aspartate transcarbamylase, and dihydroorotase complex) activation.
Proteomic study of pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome in rats.
Lin et al., Hong Kong, Hong Kong. In Chem Res Toxicol, Oct 2015
Subsequently, bioinformatic analysis suggested that two proteins, carbamoyl-phosphate synthase (CPS1) (p < 0.05) and ATP synthase subunit beta (ATP5B) (p < 0.01) were associated with RTS-L intoxication.
Protein carbonylation associated to high-fat, high-sucrose diet and its metabolic effects.
Medina et al., Vigo, Spain. In J Nutr Biochem, 2014
The increment of carbonylation by HFHS diet was singularly selective on specific target proteins: albumin from plasma and liver, and hepatic proteins such as mitochondrial carbamoyl-phosphate synthase (ammonia), mitochondrial aldehyde dehydrogenase, argininosuccinate synthetase, regucalcin, mitochondrial adenosine triphosphate synthase subunit beta, actin cytoplasmic 1 and mitochondrial glutamate dehydrogenase 1.
Molecular study on the carAB operon reveals that carB gene is required for swimming and biofilm formation in Xanthomonas citri subsp. citri.
Zou et al., Fuzhou, China. In Bmc Microbiol, 2014
BACKGROUND: The carA and carB genes code the small and large subunits of carbamoyl-phosphate synthase (CPS) that responsible for arginine and pyrimidine production.
4217C>A polymorphism in carbamoyl-phosphate synthase 1 gene may not associate with hyperammonemia development during valproic acid-based therapy.
Itoh et al., Shizuoka, Japan. In Epilepsy Res, 2014
Polymorphisms in the genes encoding carbamoyl-phosphate synthase 1 (CPS1) and N-acetylglutamate synthase (NAGS) were recently reported to be risk factors for the development of hyperammonemia during valproic acid-based therapy.
Genotyping accuracy of high-resolution DNA melting instruments.
Wittwer et al., Dalian, China. In Clin Chem, 2014
METHODS: Each genotype of the single nucleotide variant (SNV) (c.3405-29A>T) of CPS1 (carbamoyl-phosphate synthase 1, mitochondrial) was amplified by PCR in the presence of LCGreen Plus with 4 PCR product lengths.
Proteomic analysis of liver mitochondria from rats with nonalcoholic steatohepatitis.
Jin et al., Hangzhou, China. In World J Gastroenterol, 2014
Novel proteins that may be involved in NASH pathogenesis including the trifunctional enzyme Hadha, thyroxine, prohibitin, aldehyde dehydrogenase ALDH1L2, UDP-glucuronosyltransferase 2B31, and carbamoyl-phosphate synthase were identified using bioinformatics tools.
Screening and identification of ClpE interaction proteins in Streptococcus pneumoniae by a bacterial two-hybrid system and co-immunoprecipitation.
Zhang et al., Chongqing, China. In J Microbiol, 2013
We obtained eight potential ClpE interaction proteins, including carbamoyl-phosphate synthase, pyruvate oxidase (SpxB), phosphoenolpyruvate-protein phosphotransferase, aminopeptidase N (pepN), L-lactate dehydrogenase, ribosomal protein S4, sensor histidine kinase (SPD_2019), and FtsW (a cell division protein).
A20 is up-regulated in primary mouse hepatocytes subjected to hypoxia and reperfusion.
Zhang et al., Shenyang, China. In Cell Biochem Funct, 2012
Zinc finger protein A20, mercaptopyruvate sulfur transferase, apolipoprotein E precursor and carbamoyl-phosphate synthase mitochondrial precursor were identified as differentially expressed in differently exposed groups.
Human carbamoyl phosphate synthetase I (CPSI): insights on the structural role of the unknown function domains.
Azevedo et al., Porto, Portugal. In Biochem Biophys Res Commun, 2012
the human CPS unknown function domains are spatially located in a region that corresponds to the a/b subunits interface in Escherichia coli CPS. [Review]
Carbamoyl phosphate synthetase 1 deficiency in Italy: clinical and genetic findings in a heterogeneous cohort.
Morrone et al., Florence, Italy. In Gene, 2012
Mutation analysis in these patients identified 17 genetic lesions, 9 of which were new confirming their "private" nature.
Late-onset carbamoyl phosphate synthetase 1 deficiency in an adult cured by liver transplantation.
Jeffrey et al., Australia. In Liver Transpl, 2011
Case Report: Late-onset carbamoyl phosphate synthetase 1 deficiency in an adult cured by liver transplantation.
Discovery of sexual dimorphisms in metabolic and genetic biomarkers.
Illig et al., München, Germany. In Plos Genet, 2011
Sex-specific genome-wide association studies (GWAS) showed genome-wide significant differences in beta-estimates for SNPs in the CPS1 locus (carbamoyl-phosphate synthase 1, significance level: p<3.8×10(-10);
Molecular defects in human carbamoy phosphate synthetase I: mutational spectrum, diagnostic and protein structure considerations.
Summar et al., Zürich, Switzerland. In Hum Mutat, 2011
This is the first large-scale report of CPS1 mutations spanning a wide variety of molecular defects highlighting important regions in this protein.
DNA methylation suppresses expression of the urea cycle enzyme carbamoyl phosphate synthetase 1 (CPS1) in human hepatocellular carcinoma.
Liu et al., Gainesville, United States. In Am J Pathol, 2011
DNA methylation is a key mechanism of silencing CPS1 expression in human hepatocellular carcinoma cells.
New developments in the treatment of hyperammonemia: emerging use of carglumic acid.
Filippi et al., Florence, Italy. In Int J Gen Med, 2010
In some of these conditions the high blood ammonia levels are due to the reduction of N-acetylglutamate, an essential cofactor necessary for the function of the urea cycle, or to the reduction of carbamoyl-phosphate synthase-I activity.
Screening of nitrosative stress resistance genes in Coxiella burnetii: Involvement of nucleotide excision repair.
Cao et al., United States. In Microb Pathog, 2010
Among the ten candidate genes identified, the transposase, UvrB and DNA topoisomerase IV are involved in DNA transaction; the sigma-32 factor and the putative DNA-binding protein may be involved in transcriptional regulation; IF-2 is involved in protein translation; malate dehydrogenase and carbamoyl-phosphate synthase are metabolic enzymes; and the ABC transporter is a membrane-bound protein.
Contribution of CDP/Cux, a transcription factor, to cell cycle progression.
Liang et al., Suzhou, China. In Acta Biochim Biophys Sin (shanghai), 2007
The generation of CDP/Cux p110 stimulates the expression of reporter plasmid containing the promoter sequences of some S phase-specific-genes such as DNA polymerase a gene, dihydrofolate reductase gene, carbamoyl-phosphate synthase/aspartate carbamoyl-transferase/dihydroorotase gene, and cyclin A gene.
Amino acids as regulators and components of nonproteinogenic pathways.
Meijer, Amsterdam, Netherlands. In J Nutr, 2003
Glutamate also controls the rate of urea synthesis, not only as the precursor of ammonia and aspartate, but as substrate for synthesis of N-acetylglutamate, the essential activator of carbamoyl-phosphate synthase.
Nitrogenase activity and dark CO2 fixation in the lichen Peltigera aphthosa Willd.
Stewart et al., Dundee, United Kingdom. In Planta, 1981
and carbamoyl-phosphate synthase (EC and were detected but the activities of PEP carboxykinase (EC
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