Clinical features and CPS1 variants in Chinese patients with carbamoyl phosphate synthetase 1 deficiency.
Dong H, Sang T, Ma X, Song J, Chen Z, Zhang H, Jin Y, Li M, Dong D, Sun L, Zhu Z, Zhang Y, Yang Y., Free PMC Article

The Role of Carbamoyl Phosphate Synthetase 1 as a Prognostic Biomarker in Patients With Acetaminophen-induced Acute Liver Failure.
Kwan R, Chen L, Park MJ, Su Z, Weerasinghe SVW, Lee WM, Durkalski-Mauldin VL, Fontana RJ, Omary MB., Free PMC Article

Variant in the allosteric domain of CPS1 protein associated with effectiveness of N-carbamoyl glutamate therapy in neonatal onset CPS1 deficiency.
Gragnaniello V, Gueraldi D, Puma A, Commone A, Loro C, Cazzorla C, Häberle J, Burlina AB.

Integrated Proteomics and Metabolomics Analyses of Serum in Chinese Patients with Severe and Active Graves' Orbitopathy: A Cross-sectional Study.
Shi TT, Liu HY, Zhu XR, Xin Z, Hua L, Xie RR, Sun R, Cao X, Yang JK.

NAGS, CPS1, and SLC25A13 (Citrin) at the Crossroads of Arginine and Pyrimidines Metabolism in Tumor Cells.
Owusu-Ansah M, Guptan N, Alindogan D, Morizono M, Caldovic L., Free PMC Article

Ornithine aminotransferase and carbamoyl phosphate synthetase 1 involved in ammonia metabolism serve as novel targets for early stages of gastric cancer.
Jiang Z, Wei C, Luo Y, Xiao Y, Wang L, Guo W, Yuan X., Free PMC Article

O-GlcNAcylation enhances CPS1 catalytic efficiency for ammonia and promotes ureagenesis.
Soria LR, Makris G, D'Alessio AM, De Angelis A, Boffa I, Pravata VM, Rüfenacht V, Attanasio S, Nusco E, Arena P, Ferenbach AT, Paris D, Cuomo P, Motta A, Nitzahn M, Lipshutz GS, Martínez-Pizarro A, Richard E, Desviat LR, Häberle J, van Aalten DMF, Brunetti-Pierri N., Free PMC Article

The Role of TRIP6, ABCC3 and CPS1 Expression in Resistance of Ovarian Cancer to Taxanes.
Seborova K, Kloudova-Spalenkova A, Koucka K, Holy P, Ehrlichova M, Wang C, Ojima I, Voleska I, Daniel P, Balusikova K, Jelinek M, Kovar J, Rob L, Hruda M, Mrhalova M, Soucek P, Vaclavikova R., Free PMC Article

Unfavorable clinical outcomes in patients with carbamoyl phosphate synthetase 1 deficiency.
Choi Y, Oh A, Lee Y, Kim GH, Choi JH, Yoo HW, Lee BH.

Discovery of a Carbamoyl Phosphate Synthetase 1-Deficient HCC Subtype With Therapeutic Potential Through Integrative Genomic and Experimental Analysis.
Wu T, Luo G, Lian Q, Sui C, Tang J, Zhu Y, Zheng B, Li Z, Zhang Y, Zhang Y, Bao J, Hu J, Shen S, Yang Z, Wu J, Wang K, Zhao Y, Yang S, Wang S, Qiu X, Wang W, Wu X, Wang H, Gu J, Chen L.

Variants associated with urea cycle disorders in Japanese patients: Nationwide study and literature review.
Kido J, Matsumoto S, Sugawara K, Sawada T, Nakamura K.

Mitochondrial dysfunction as a mechanistic biomarker in patients with non-alcoholic fatty liver disease (NAFLD).
Ajaz S, McPhail MJ, Gnudi L, Trovato FM, Mujib S, Napoli S, Carey I, Agarwal K.

Association between genetic variations in carbamoyl-phosphate synthetase gene and persistent neonatal pulmonary hypertension.
El-Khazragy N, El Barbary M, Fouad H, Abdelgawad A, Rabie D.

Suppression of the NTS-CPS1 regulatory axis by AFF1 in lung adenocarcinoma cells.
Yue J, Dai Q, Hao S, Zhu S, Liu X, Tang Z, Li M, Fang H, Lin C, Luo Z., Free PMC Article

CPS1: Looking at an ancient enzyme in a modern light.
Nitzahn M, Lipshutz GS., Free PMC Article

The Application of Next-Generation Sequencing (NGS) in Neonatal-Onset Urea Cycle Disorders (UCDs): Clinical Course, Metabolomic Profiling, and Genetic Findings in Nine Chinese Hyperammonemia Patients.
Zhou Q, Huang H, Ma L, Zhu T., Free PMC Article

Neonatal factors related to survival and intellectual and developmental outcome of patients with early-onset urea cycle disorders.
Pontoizeau C, Roda C, Arnoux JB, Vignolo-Diard P, Brassier A, Habarou F, Barbier V, Grisel C, Abi-Warde MT, Boddaert N, Kuster A, Servais A, Kaminska A, Hennequin C, Dupic L, Lesage F, Touati G, Valayannopoulos V, Chadefaux-Vekemans B, Oualha M, Eisermann M, Ottolenghi C, de Lonlay P.

Molecular, biochemical, and clinical analyses of five patients with carbamoyl phosphate synthetase 1 deficiency.
Fan L, Zhao J, Jiang L, Xie L, Ma J, Li X, Cheng M., Free PMC Article

Non-alcoholic fatty liver disease alters expression of genes governing hepatic nitrogen conversion.
Eriksen PL, Vilstrup H, Rigbolt K, Suppli MP, Sørensen M, Heebøll S, Veidal SS, Knop FK, Thomsen KL.

Caspase recruitment domain family member 10 regulates carbamoyl phosphate synthase 1 and promotes cancer growth in bladder cancer cells.
Liu X, Zhang X, Bi J, Li Z, Zhang Z, Kong C., Free PMC Article

Expression and clinical significance of CPS1 in glioblastoma multiforme.
Wu G, Yan Y, Zhou Y, Wang X, Wei J, Chen X, Lin W, Ou C, Zhou J, Xu Z.

A liver-humanized mouse model of carbamoyl phosphate synthetase 1-deficiency.
Srinivasan RC, Zabulica M, Hammarstedt C, Wu T, Gramignoli R, Kannisto K, Ellis E, Karadagi A, Fingerhut R, Allegri G, Rüfenacht V, Thöny B, Häberle J, Nuoffer JM, Strom SC.

Overexpression of carbamoyl-phosphate synthase 1 significantly improves ureagenesis of human liver HepaRG cells only when cultured under shaking conditions.
Adam AAA, van der Mark VA, Ruiter JPN, Wanders RJA, Oude Elferink RPJ, Chamuleau RAFM, Hoekstra R.

Hepatocyte Antigen Expression in Barrett Esophagus and Associated Neoplasia.
Shah SS, Chandan VS.

Conditional disruption of hepatic carbamoyl phosphate synthetase 1 in mice results in hyperammonemia without orotic aciduria and can be corrected by liver-directed gene therapy.
Khoja S, Nitzahn M, Hermann K, Truong B, Borzone R, Willis B, Rudd M, Palmer DJ, Ng P, Brunetti-Pierri N, Lipshutz GS., Free PMC Article