glycocyamine has been researched along with Argininosuccinic Aciduria in 2 studies
glycocyamine: RN given refers to parent cpd; structure
guanidinoacetate : A monocarboxylic acid anion that is the conjugate base of guanidinoacetic acid, obtained by deprotonation of the carboxy group.
guanidinoacetic acid : The N-amidino derivative of glycine.
Argininosuccinic Aciduria: Rare autosomal recessive disorder of the urea cycle which leads to the accumulation of argininosuccinic acid in body fluids and severe HYPERAMMONEMIA. Clinical features of the neonatal onset of the disorder include poor feeding, vomiting, lethargy, seizures, tachypnea, coma, and death. Later onset results in milder set of clinical features including vomiting, failure to thrive, irritability, behavioral problems, or psychomotor retardation. Mutations in the ARGININOSUCCINATE LYASE gene cause the disorder.
| Excerpt | Relevance | Reference |
|---|---|---|
| "MR spectroscopy in a patient with argininosuccinate lyase deficiency revealed elevated cerebral guanidinoacetate signals, indicating that the phenomenon of increased levels of this compound in brain tissue is not limited to creatine deficiencies." | 1.33 | Cerebral 1H MR spectroscopy showing elevation of brain guanidinoacetate in argininosuccinate lyase deficiency. ( Oudkerk, M; Reijngoud, DJ; Sijens, PE; Soorani-Lunsing, RJ; van Spronsen, FJ, 2006) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 2 (100.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Sijens, PE | 2 |
| Reijngoud, DJ | 2 |
| Soorani-Lunsing, RJ | 2 |
| Oudkerk, M | 1 |
| van Spronsen, FJ | 2 |
| Verhoeven, NM | 1 |
| Jakobs, C | 1 |
2 other studies available for glycocyamine and Argininosuccinic Aciduria
| Article | Year |
|---|---|
Cerebral 1H MR spectroscopy showing elevation of brain guanidinoacetate in argininosuccinate lyase deficiency.
Topics: Amino Acid Metabolism, Inborn Errors; Argininosuccinic Aciduria; Brain Chemistry; Child, Preschool; | 2006 |
High cerebral guanidinoacetate and variable creatine concentrations in argininosuccinate synthetase and lyase deficiency: implications for treatment?
Topics: Amino Acid Metabolism, Inborn Errors; Arginine; Argininosuccinate Synthase; Argininosuccinic Aciduri | 2006 |