Keywords: CPS1; Carbamoyl phosphate synthetase 1; Carbamoyl phosphate; synthetase deficiency; Gene therapy;
مقالات ISI (ترجمه نشده)
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Keywords: AhR; aryl hydrocarbon receptor; XRE; xenobiotic response element; hcit; homocitrulline; NC-XRE; non-consensus xenobiotic response element; TCDD; 2,3,7,8-tetrachlorodibenzo-p-dioxin; KLF6; Krüppel-like factor 6; CPS1; carbamoyl phosphate synthase 1;
Keywords: Urea cycle disorders; Glycerol phenylbutyrate; Children; Infants; Ammonia; Glutamine; ARG1; arginase 1; ASL; argininosuccinate lyase; ASS; argininosuccinate synthetase; BMI; body mass index; BSA; body surface area; CDC; Centers for Disease Control and Pre
Keywords: AceCS2; acetyl-CoA synthetase 2; ATP; adenosine triphosphate; AMPK; AMP-activated protein kinase; ANT2/ANT3; adenine nucleotide translocase 2 and 3; BMI; body mass index; CtBP; C-terminal-binding protein; CPS1; carbamoyl phosphate synthetase 1; CR; calori
Keywords: LPS; lipopolysaccharide; AQP; aquaporin; IMM; inner mitochondrial membranes; mtAQP8; mitochondrial aquaporin-8; CPS1; carbamyl phosphate synthetase 1; OTC; ornithine transcarbamylase; Llipopolysaccharide; Urea synthesis; Mitochondrial ammonia transport; A
Keywords: CBS; cystathionine beta synthase; CPS1; carbamoyl-phosphate synthase 1; CUBN; cubilin; DPEP1; dipeptidase 1; FUT 2; fucosyltransferase 2; GABA; gamma-amino butyric acid; GTPB10; GTP-binding protein 10; HIS; International Headache Society; HNF1A; hepatocyt
Keywords: AceCS2; acetyl-CoA synthetase 2; ADP; adenosine diphosphate; CPS1; carbamoyl phosphate synthetase 1; CPT1; carnitine palmitoyltransferase I; CR; calorie restriction; FOXO3a; forkhead box 03a; GDH; glutamate dehydrogenase; HeLa; human cervical carcinoma ce
Farnesoid X Receptor Activation Promotes Hepatic Amino Acid Catabolism and Ammonium Clearance in Mice
Keywords: Liver Proteome; INT-747; Cps1; Glutamine Synthetase; BA; bile acid; ChIP; chromatin immunoprecipitation; Cps1; carbamoyl phosphate synthetase-1; FXR; Farnesoid X receptor; OCA; obeticholic acid; qPCR; quantitative polymerase chain reaction; TSS; transcrip
Profiles of amino acids and biogenic amines in the plasma of Cri-du-Chat patients
Keywords: 5-HT; serotonin; 5p; chromosome 5; AcOrn; acetyl ornitine; ADMA; dimethylarginine asymmetric; ADP; adenosine diphosphate; AGAT; glycine amidinotransferase; Ala; alanine; Alpha AAA; alpha-aminoadipic acid; Arg; arginine; ASL; argininosuccinate lyase; Asn;
Vestiges of Ent3p/Ent5p function in the giardial epsin homolog
Keywords: GlENTHp; Giardia lamblia ENTH protein; CCVs; clathrin-coated vesicles; ENTH; epsin N-terminal homology domain; ANTH; AP180 N-terminal homology domain; VHS; Vps27, Hrs and STAM domain; UIMs; ubiquitin-interaction motifs; epsinR; epsin related; AP-1; adapto
Fatal hyperammonemia and carbamoyl phosphate synthetase 1 (CPS1) deficiency following high-dose chemotherapy and autologous hematopoietic stem cell transplantation
Keywords: 3-OH-B; 3-Hydroxy-butyrate; AcAc; Acetoacetate; BW; Bodyweight; CA VA; Carbonic anhydrase VA; CPS1; Carbamoyl phosphate synthetase 1; HSCT; Hematopoietic stem cell transplantation; NAGS; N-acetyl-glutamate synthase; NCG; N-carbamyl-glutamate; OTC; Ornithi
Acute and sub-acute oral toxicological evaluations and mutagenicity of N-carbamylglutamate (NCG)
Keywords: N-carbamylglutamate; Toxicology; Acute/subacute toxicity; Mutagenicity; NCG; N-carbamylglutamate; NAG; N-acetylglutamate; CPS1; carbamyl phosphate synthetase 1; Arg; arginine; NAGS; NAG synthase; GH; growth hormone; SD; Sprague-Dawley; BMM; bone marrow
Glutathione metabolism links FOXRED1 to NADH:ubiquinone oxidoreductase (complex I) deficiency: A hypothesis
Keywords: ABC; ATP-binding cassette; ADH3; alcohol dehydrogenase 3; ALDH; aldehyde dehydrogenase; CFTR; cystic fibrosis transmembrane conductance regulator; CPS1; carbamoyl phosphate synthase; DMGDH; dimethylglycine dehydrogenase; DHF; dihydrofolate; DMGO; dimethyl
Augmenting Ureagenesis in Patients with Partial Carbamyl Phosphate Synthetase 1 Deficiency with N-carbamyl-l-glutamate
Keywords: CPS1; Carbamyl phosphate synthetase 1; NAGS; N-Acetyl-l-glutamate synthase; NCG; N-Carbamyl-l-glutamate;
Peptide tyrosine tyrosine levels are increased in patients with urea cycle disorders
Keywords: ASL; Argininosuccinate lyase; ASS1; Argininosuccinate synthase 1; BMI; Body mass index; CPS1; Carbamoyl-phosphate synthase 1; NAGS; N-acetylglutamate synthase; OTC; Ornithine carbamoyltransferase; OTCD; Ornithine carbamoyltransferase deficiency; PYY; Pept
Amino acid disorders detected by quantitative amino acid HPLC analysis in Thailand: An eight-year experience
Keywords: ASL; argininosuccinate lyase; ASS; argininosuccinate synthetase; CPS1; carbamoylphosphate synthetase I; HCY; homocystinuria; HPA; hyperphenylalaninemia; HPLC; high-performance liquid chromatography; IEM; inborn error of metabolism; MSUD; maple syrup urine
Early Clinical Manifestations and Eating Patterns in Patients with Urea Cycle Disorders
Keywords: ASL; Argininosuccinic acid lyase; ASS; Argininosuccinic acid synthetase; CPS1; Carbamoyl phosphate synthetase 1; OTC; Ornithine transcarbamylase; UCD; Urea cycle disorder;
SIRT5 deacetylates and activates urate oxidase in liver mitochondria of mice
Keywords: UOX; urate oxidase; SIRT5 Tg; SIRT5-overexpressing transgenic; CPS1; carbamoyl phosphate synthetase 1; SIRT5; Urate oxidase; Mouse; Liver; Mitochondria;
A novel biochemically salvageable animal model of hyperammonemia devoid of N-acetylglutamate synthase
Keywords: CPS1; Carbamyl phosphate synthetase 1; NAG; N-acetyl-l-glutamate; NAGS; N-acetylglutamate synthase; NCG; N-carbamyl-l-glutamate; Cit; Citrulline; KOMP; Knock-out Mouse Project; OTC; Ornithine transcarbamylase; Hyperammonemia; Urea cycle; N-acetylglutamate
Regulation and protection of mitochondrial physiology by sirtuins
Keywords: Acetyl-CoA; acetyl-coenzyme A; AceCS2; acetyl-coenzyme A synthethase 2; ACC; Acetyl-CoA carboxylase; ADP; adenosine diphosphate; AIF; apoptosis-inducing factor; AMPK; 5â² adenosine monophosphate-activated protein kinase; ANT; adenine nucleotide transloca
Ammonium metabolism in humans
Keywords: NH3; ammonia; NH4+; ammonium ions; AST; aspartate aminotransferase; ALT; alanine aminotransferase; BCAT; branched-chain amino acids aminotransferase; KGA; kidney-type glutaminase; GAC; glutaminase C; GAM; glutaminase M; GDH1; glutamate dehydrogenase-1; GD
Embryonic Ductal Plate Cells Give Rise to Cholangiocytes, Periportal Hepatocytes, and Adult Liver Progenitor Cells
Keywords: Hepatic Cell Differentiation; Liver Development; Liver Regeneration; CEACAM; carcinoembryonic antigen-related cell adhesion molecule; CDE; choline-deficient ethionine-supplemented; CK19; cytokeratin 19; CPS1; carbamoyl-phosphate-synthetase 1; CreER; cyc
Induction of a mature hepatocyte phenotype in adult liver derived progenitor cells by ectopic expression of transcription factors
Keywords: ALDPC; adult liver-derived progenitor cells; iPS; induced pluripotent stem cells; LETF; liver enriched transcription factors; LD; liver differentiation medium; HBM; hepatocyte basal medium; MM; maintenance medium; ESC; embryonic stem cells; Foxa2; forkhea
Overexpression of SIRT5 confirms its involvement in deacetylation and activation of carbamoyl phosphate synthetase 1
Keywords: SIR2; silent information regulator 2; CPS1; carbamoyl phosphate synthetase 1; Tg; transgenic; KO; knockout; SIRT5; Mitochondria; Urea cycle; Liver;