guanidine has been researched along with Hyperargininemia in 1 studies
Guanidine: A strong organic base existing primarily as guanidium ions at physiological pH. It is found in the urine as a normal product of protein metabolism. It is also used in laboratory research as a protein denaturant. (From Martindale, the Extra Pharmacopoeia, 30th ed and Merck Index, 12th ed) It is also used in the treatment of myasthenia and as a fluorescent probe in HPLC.
guanidine : An aminocarboxamidine, the parent compound of the guanidines.
Hyperargininemia: A rare autosomal recessive disorder of the urea cycle. It is caused by a deficiency of the hepatic enzyme ARGINASE. Arginine is elevated in the blood and cerebrospinal fluid, and periodic HYPERAMMONEMIA may occur. Disease onset is usually in infancy or early childhood. Clinical manifestations include seizures, microcephaly, progressive mental impairment, hypotonia, ataxia, spastic diplegia, and quadriparesis. (From Hum Genet 1993 Mar;91(1):1-5; Menkes, Textbook of Child Neurology, 5th ed, p51)
| Excerpt | Relevance | Reference |
|---|---|---|
| "Hyperargininemia is caused by deficiency of arginase 1, which catalyzes the hydrolysis of L-arginine to urea as the final enzyme in the urea cycle." | 8.91 | Hyperargininemia due to arginase I deficiency: the original patients and their natural history, and a review of the literature. ( Ensenauer, R; Häussinger, D; Mayatepek, E; Schlune, A; Vom Dahl, S, 2015) |
| "Hyperargininemia is caused by deficiency of arginase 1, which catalyzes the hydrolysis of L-arginine to urea as the final enzyme in the urea cycle." | 4.91 | Hyperargininemia due to arginase I deficiency: the original patients and their natural history, and a review of the literature. ( Ensenauer, R; Häussinger, D; Mayatepek, E; Schlune, A; Vom Dahl, S, 2015) |
| 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 |
|---|---|
| Schlune, A | 1 |
| Vom Dahl, S | 1 |
| Häussinger, D | 1 |
| Ensenauer, R | 1 |
| Mayatepek, E | 1 |
1 review available for guanidine and Hyperargininemia
| Article | Year |
|---|---|
Hyperargininemia due to arginase I deficiency: the original patients and their natural history, and a review of the literature.
Topics: Arginase; Arginine; Child, Preschool; Female; Guanidine; Humans; Hyperammonemia; Hyperargininemia; I | 2015 |