alpha-ketoglutaramate has been researched along with Urea Cycle Disorders, Inborn in 1 studies
alpha-ketoglutaramate: deaminated metabolite of glutamine in csf of patients with hepatic coma; intermediate in the detoxification of ammonia in brain; structure
Urea Cycle Disorders, Inborn: Rare congenital metabolism disorders of the urea cycle. The disorders are due to mutations that result in complete (neonatal onset) or partial (childhood or adult onset) inactivity of an enzyme, involved in the urea cycle. Neonatal onset results in clinical features that include irritability, vomiting, lethargy, seizures, NEONATAL HYPOTONIA; RESPIRATORY ALKALOSIS; HYPERAMMONEMIA; coma, and death. Survivors of the neonatal onset and childhood/adult onset disorders share common risks for ENCEPHALOPATHIES, METABOLIC, INBORN; and RESPIRATORY ALKALOSIS due to HYPERAMMONEMIA.
| 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 | 1 (100.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Cooper, AJ | 1 |
| Kuhara, T | 1 |
1 review available for alpha-ketoglutaramate and Urea Cycle Disorders, Inborn
| Article | Year |
|---|---|
α-Ketoglutaramate: an overlooked metabolite of glutamine and a biomarker for hepatic encephalopathy and inborn errors of the urea cycle.
Topics: Amidohydrolases; Aminohydrolases; Ammonia; Animals; Biomarkers; Carbon; Glutamine; Hepatic Encephalo | 2014 |