creatine has been researched along with Argininosuccinic Aciduria in 3 studies
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 |
|---|---|---|
| " The goal of this study was to understand the effect on the known toxic metabolite ASA and the assumed toxic metabolite guanidinosuccinic acid (GSA) on developing brain cells, and to evaluate the potential role of creatine (Cr) supplementation, as it was described protective for brain cells exposed to ammonia." | 2.61 | Argininosuccinate neurotoxicity and prevention by creatine in argininosuccinate lyase deficiency: An in vitro study in rat three-dimensional organotypic brain cell cultures. ( Braissant, O; Diez-Fernandez, C; Diserens, G; Häberle, J; Henry, H; Hertig, D; Loup, M; Nuoffer, JM; Vermathen, P, 2019) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 2 (66.67) | 29.6817 |
| 2010's | 1 (33.33) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Diez-Fernandez, C | 1 |
| Hertig, D | 1 |
| Loup, M | 1 |
| Diserens, G | 1 |
| Henry, H | 1 |
| Vermathen, P | 1 |
| Nuoffer, JM | 1 |
| Häberle, J | 2 |
| Braissant, O | 1 |
| van Spronsen, FJ | 1 |
| Reijngoud, DJ | 1 |
| Verhoeven, NM | 1 |
| Soorani-Lunsing, RJ | 1 |
| Jakobs, C | 1 |
| Sijens, PE | 1 |
| Roze, E | 1 |
| Azuar, C | 1 |
| Menuel, C | 1 |
| Guillevin, R | 1 |
1 review available for creatine and Argininosuccinic Aciduria
| Article | Year |
|---|---|
Argininosuccinate neurotoxicity and prevention by creatine in argininosuccinate lyase deficiency: An in vitro study in rat three-dimensional organotypic brain cell cultures.
Topics: Animals; Argininosuccinic Acid; Argininosuccinic Aciduria; Brain; Cells, Cultured; Creatine; Humans; | 2019 |
2 other studies available for creatine and Argininosuccinic Aciduria
| Article | Year |
|---|---|
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 |
Usefulness of magnetic resonance spectroscopy in urea cycle disorders.
Topics: Adult; Argininosuccinic Aciduria; Brain Diseases, Metabolic; Creatine; Female; Humans; Magnetic Reso | 2007 |