Carbamyl Phosphate (Carbamoyl Phosphate)

The monoanhydride of carbamic acid with PHOSPHORIC ACID. It is an important intermediate metabolite and is synthesized enzymatically by CARBAMYL-PHOSPHATE SYNTHASE (AMMONIA) and CARBAMOYL-PHOSPHATE SYNTHASE (GLUTAMINE-HYDROLYZING).

Also Known As:

Networked: 234 relevant articles (2 outcomes, 14 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Häberle, Johannes: 8 articles (11/2021 - 12/2014)
2.	Kido, Jun: 5 articles (11/2021 - 08/2014)
3.	Nakamura, Kimitoshi: 5 articles (11/2021 - 08/2014)
4.	Matsumoto, Shirou: 4 articles (11/2021 - 09/2017)
5.	Khoja, Suhail: 4 articles (01/2020 - 01/2018)
6.	Lipshutz, Gerald S: 4 articles (01/2020 - 01/2018)
7.	Rüfenacht, Véronique: 4 articles (11/2019 - 12/2014)
8.	Nitzahn, Matthew: 3 articles (01/2020 - 11/2019)
9.	Truong, Brian: 3 articles (01/2020 - 01/2018)
10.	Gropman, Andrea L: 3 articles (01/2016 - 01/2010)

Related Diseases

1.	Coma (Comas) 09/01/1992 - "Carbamyl phosphate synthetase-1 deficiency discovered after valproic acid-induced coma." 01/01/1985 - "Carbamyl phosphate synthetase I deficiency (CPSD) is an autosomal recessive disorder of ureagenesis characterized by hyperammonemic coma in the neonatal period. " 08/01/2000 - "This is the first case of fulminant neonatal-onset carbamoyl-phosphate synthase I deficiency treated by continuous hemodiafiltration indicating that this is an available and effective procedure for neonates with hyperammonemic coma." 07/01/1996 - "A child with carbamyl-phosphate-synthetase defect who died after prolonged continuous hemodiafiltration in deep coma proved to have high aminoacid losses despite aminoacid infusion. " 12/01/1991 - "The therapy was effective in avoiding neonatal hyperammonemic coma and death in seven patients with carbamoyl phosphate synthetase deficiency, argininosuccinate synthetase deficiency, and argininosuccinate lyase deficiency. "
2.	Argininosuccinic Aciduria 12/01/1999 - "We report here our experience in the long-term management of 28 patients with citrullinaemia, 13 patients with carbamoyl phosphate synthase deficiency and 15 patients with argininosuccinic aciduria. " 01/01/2021 - "Sequence analysis revealed nine diagnostic variants, including three novel ones, which caused Argininosuccinic aciduria (ASA) in one case, Carbamoyl phosphate synthetase 1deficiency (CPS1D) in two cases, Ornithine transcarbamylase deficiency (OTCD) in one case, and Citrin deficiency in 1case. " 12/01/2008 - "High carrier frequencies for cerebrotendinous xanthomatosis, prolidase deficiency, argininosuccinate lyase deficiency, and carbamyl phosphate synthetase I deficiency were identified as 1:11, 1:21, 1:41, and 1:95, respectively. " 12/01/1991 - "The therapy was effective in avoiding neonatal hyperammonemic coma and death in seven patients with carbamoyl phosphate synthetase deficiency, argininosuccinate synthetase deficiency, and argininosuccinate lyase deficiency. " 12/01/2008 - "Multiple patients with four rare and severe inborn errors of metabolism cerebrotendinous xanthomatosis, prolidase deficiency, argininosuccinate lyase deficiency, and carbamyl phosphate synthetase I deficiency were identified in an isolated Druze village in northern Israel. "
3.	Citrullinemia 11/01/2021 - "Of them, a total of 78 patients with UCDs (30 male and 16 female ornithine transcarbamylase deficiency (OTCD), 21 carbamoyl phosphate synthetase 1 deficiency (CPSD), 10 argininosuccinate synthetase deficiency (ASSD) and 1 arginase 1 deficiency (ARGD)) had undergone LT. " 09/01/2017 - "Among these 177 patients, 42 (seven with carbamoyl phosphate synthetase 1 deficiency, 27 with ornithine transcarbamylase deficiency, seven with argininosuccinate synthetase deficiency, and one with arginase 1 deficiency) underwent living-donor LT. " 12/01/1991 - "The therapy was effective in avoiding neonatal hyperammonemic coma and death in seven patients with carbamoyl phosphate synthetase deficiency, argininosuccinate synthetase deficiency, and argininosuccinate lyase deficiency. " 03/01/1992 - "Hyperammonemia, abnormalities in plasma amino acids and abnormalities of standard liver functions were corrected by orthotopic liver transplantation in a 14-day-old boy with carbamyl phosphate synthetase-I deficiency and in a 35-yr-old man with argininosuccinic acid synthetase deficiency. " 06/01/2021 - "A total of 229 patients with UCDs (126 patients: ornithine transcarbamylase deficiency [OTCD]; 33: carbamoyl phosphate synthetase 1 deficiency [CPS1D]; 48: argininosuccinate synthetase deficiency [ASSD]; 14: argininosuccinate lyase deficiency [ASLD]; and 8: arginase 1 deficiency [ARG1D]) were enrolled in the present study. "
4.	Ornithine Carbamoyltransferase Deficiency Disease 10/08/2020 - "Ornithine transcarbamylase deficiency (OTCD) and carbamoyl phosphate synthetase 1 deficiency (CPS1D) are screened in six and eight states in the United States. " 01/01/2012 - "Noncirrhotic NPCs derived from patients with carbamyl phosphate synthetase and ornithine transcarbamylase deficiencies were used as controls. " 10/01/2004 - "We report on 5 children with UCDs, ie, 2 male patients with X-linked ornithine transcarbamylase deficiency and 2 male patients with carbamoyl phosphate synthase deficiency, all of whom had neonatal presentations and underwent orthotopic liver transplantation before 1 year of age, and 1 female patient with partial X-linked ornithine transcarbamylase deficiency that was intractable to medical therapy, who underwent transplantation at 35 months of age. " 03/01/1992 - "Seven other patients--six with ornithine transcarbamylase deficiency and another with carbamyl phosphate synthetase-I deficiency--are known to have been treated elsewhere with liver transplantation 1 1/2 yr or longer ago. " 12/01/1991 - "Two patients with carbamoyl phosphate synthetase deficiency and two with ornithine transcarbamylase deficiency have subsequently died; three additional patients with the latter disorder have received orthotopic liver transplants. "
5.	Neoplasms (Cancer) 12/01/2019 - "Caspase recruitment domain family member 10 regulates carbamoyl phosphate synthase 1 and promotes cancer growth in bladder cancer cells." 01/01/2023 - "High tumor expression of carbamoyl phosphate synthase 1 (CPS1) was strongly linked to the presence of LKB1 mutations, was the best predictor of NSCLC dexamethasone (DEX) sensitivity (p < 10-16) but was not mechanistically involved in DEX sensitivity. " 01/01/2018 - "This warrants additional investigation to look for the possible role of carbamoyl phosphate synthetase 1 in the pathogenesis of Barrett-related neoplasia." 04/24/1981 - "Carbamoyl phosphate synthetase (glutamine-hydrolyzing): increased activity in cancer cells." 08/01/1980 - "The apparent Ki of PALA for leukocyte ATCase with carbamoyl phosphate as the variable substrate is 5 nM. Uptake of PALA into leukocytes in vitro is saturable and occurs at a moderate rate comparable to that measured in murine tumor cells. "

Related Drugs and Biologics

1.	Ligases (Synthetase)
2.	Ammonia
3.	Glutamine (L-Glutamine)
4.	Glutamic Acid (Glutamate)
5.	Enzymes
6.	Urea (Carbamide)
7.	Ornithine
8.	Biomarkers (Surrogate Marker)
9.	Ornithine Carbamoyltransferase (Ornithine Transcarbamylase)
10.	Aspartate Carbamoyltransferase

Related Therapies and Procedures

1.	Therapeutics
2.	Liver Transplantation
3.	Hemodiafiltration
4.	Transplantation
5.	Injections