Page last updated: 2024-10-17

alpha-keto-delta-guanidinovaleric acid and Hyperargininemia

alpha-keto-delta-guanidinovaleric acid has been researched along with Hyperargininemia in 1 studies

5-guanidino-2-oxopentanoic acid : A 2-oxo monocarboxylic acid that is 2-oxopentanoic acid in which one of the methyl hydrogens is substituted by a carbamimidamido group.

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)

Research

Studies (1)

TimeframeStudies, this research(%)All Research%
pre-19901 (100.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Marescau, B1
Lowenthal, A1
Terheggen, HG1
Esmans, E1
Alderweireldt, F1

Other Studies

1 other study available for alpha-keto-delta-guanidinovaleric acid and Hyperargininemia

ArticleYear
Guanidino compounds in hyperargininemia.
    Advances in experimental medicine and biology, 1982, Volume: 153

    Topics: Amino Acid Metabolism, Inborn Errors; Arginine; Chromatography, Liquid; Chromatography, Thin Layer;

1982