urea has been researched along with Propionic Acidemia in 5 studies
pseudourea: clinical use; structure
isourea : A carboximidic acid that is the imidic acid tautomer of urea, H2NC(=NH)OH, and its hydrocarbyl derivatives.
Propionic Acidemia: Autosomal recessive metabolic disorder caused by mutations in PROPIONYL-COA CARBOXYLASE genes that result in dysfunction of branch chain amino acids and of the metabolism of certain fatty acids. Neonatal clinical onset is characterized by severe metabolic acidemia accompanied by hyperammonemia, HYPERGLYCEMIA, lethargy, vomiting, HYPOTONIA; and HEPATOMEGALY. Survivors of the neonatal onset propionic acidemia often show developmental retardation, and intolerance to dietary proteins. Late-onset form of the disease shows mild mental and/or developmental retardation, sometimes without metabolic acidemia.
| Excerpt | Relevance | Reference |
|---|---|---|
| "The objective of this study was to determine whether N-carbamylglutamate (NCG) reduces plasma levels of ammonia and glutamine and increases the rate of ureagenesis in patients with propionic acidemia (PA)." | 9.14 | N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia. ( Ah Mew, N; Daikhin, Y; McCarter, R; Nissim, I; Tuchman, M; Yudkoff, M, 2010) |
| "The objective of this study was to determine whether N-carbamylglutamate (NCG) reduces plasma levels of ammonia and glutamine and increases the rate of ureagenesis in patients with propionic acidemia (PA)." | 5.14 | N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia. ( Ah Mew, N; Daikhin, Y; McCarter, R; Nissim, I; Tuchman, M; Yudkoff, M, 2010) |
| "Life-threatening hyperammonemia occurs in both inherited and acquired liver diseases affecting ureagenesis, the main pathway for detoxification of neurotoxic ammonia in mammals." | 4.12 | O-GlcNAcylation enhances CPS1 catalytic efficiency for ammonia and promotes ureagenesis. ( Arena, P; Attanasio, S; Boffa, I; Brunetti-Pierri, N; Cuomo, P; D'Alessio, AM; De Angelis, A; Desviat, LR; Ferenbach, AT; Häberle, J; Lipshutz, GS; Makris, G; Martínez-Pizarro, A; Motta, A; Nitzahn, M; Nusco, E; Paris, D; Pravata, VM; Richard, E; Rüfenacht, V; Soria, LR; van Aalten, DMF, 2022) |
| "Propionic acidemia is an inborn error of metabolism caused by a deficiency in the mitochondrial enzyme propionyl-CoA carboxylase that converts the propionyl CoA to methyl malonyl CoA." | 1.72 | Interorgan amino acid interchange in propionic acidemia: the missing key to understanding its physiopathology. ( Arrieta, F; Belanger-Quintana, A; Del Valle, M; Fernandez-Felix, BM; Garcia, F; Martinez-Pardo, M; Ruiz-Sala, P; Stanescu, S, 2022) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 3 (60.00) | 24.3611 |
| 2020's | 2 (40.00) | 2.80 |
| Authors | Studies |
|---|---|
| Stanescu, S | 1 |
| Belanger-Quintana, A | 1 |
| Fernandez-Felix, BM | 1 |
| Ruiz-Sala, P | 1 |
| Del Valle, M | 1 |
| Garcia, F | 1 |
| Arrieta, F | 1 |
| Martinez-Pardo, M | 1 |
| Soria, LR | 1 |
| Makris, G | 1 |
| D'Alessio, AM | 1 |
| De Angelis, A | 1 |
| Boffa, I | 1 |
| Pravata, VM | 1 |
| Rüfenacht, V | 1 |
| Attanasio, S | 1 |
| Nusco, E | 1 |
| Arena, P | 1 |
| Ferenbach, AT | 1 |
| Paris, D | 1 |
| Cuomo, P | 1 |
| Motta, A | 1 |
| Nitzahn, M | 1 |
| Lipshutz, GS | 1 |
| Martínez-Pizarro, A | 1 |
| Richard, E | 1 |
| Desviat, LR | 1 |
| Häberle, J | 1 |
| van Aalten, DMF | 1 |
| Brunetti-Pierri, N | 1 |
| Chapman, KA | 1 |
| Collado, MS | 1 |
| Figler, RA | 1 |
| Hoang, SA | 1 |
| Armstrong, AJ | 1 |
| Cui, W | 1 |
| Purdy, M | 1 |
| Simmers, MB | 1 |
| Yazigi, NA | 1 |
| Summar, ML | 1 |
| Wamhoff, BR | 1 |
| Dash, A | 1 |
| Ah Mew, N | 1 |
| McCarter, R | 1 |
| Daikhin, Y | 1 |
| Nissim, I | 1 |
| Yudkoff, M | 1 |
| Tuchman, M | 1 |
| Scholl-Bürgi, S | 1 |
| Sass, JO | 1 |
| Zschocke, J | 1 |
| Karall, D | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Increasing Ureagenesis in Inborn Errors of Metabolism With N-Carbamylglutamate[NCT01341379] | Phase 2 | 0 participants (Actual) | Interventional | 2010-12-31 | Withdrawn (stopped due to Lack of funding) | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
1 review available for urea and Propionic Acidemia
| Article | Year |
|---|---|
Amino acid metabolism in patients with propionic acidaemia.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Ammonia; Citric Acid Cycle; Diet; | 2012 |
1 trial available for urea and Propionic Acidemia
| Article | Year |
|---|---|
N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia.
Topics: Administration, Oral; Adolescent; Ammonia; Blood Chemical Analysis; Child; Child, Preschool; Confide | 2010 |
3 other studies available for urea and Propionic Acidemia
| Article | Year |
|---|---|
Interorgan amino acid interchange in propionic acidemia: the missing key to understanding its physiopathology.
Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Glutamine; Humans; Hyperammonem | 2022 |
O-GlcNAcylation enhances CPS1 catalytic efficiency for ammonia and promotes ureagenesis.
Topics: Acetylglucosamine; Ammonia; Animals; Biocatalysis; Carbamoyl-Phosphate Synthase (Ammonia); Disease M | 2022 |
Recapitulation of metabolic defects in a model of propionic acidemia using patient-derived primary hepatocytes.
Topics: Actins; Amino Acids, Branched-Chain; Ammonia; Carbon-Carbon Ligases; Cells, Cultured; Child; Fibrobl | 2016 |