Page last updated: 2024-11-02

4-phenylbutyric acid and Hyperargininemia

4-phenylbutyric acid has been researched along with Hyperargininemia in 1 studies

4-phenylbutyric acid: RN refers to the parent cpd
4-phenylbutyric acid : A monocarboxylic acid the structure of which is that of butyric acid substituted with a phenyl group at C-4. It is a histone deacetylase inhibitor that displays anticancer activity. It inhibits cell proliferation, invasion and migration and induces apoptosis in glioma cells. It also inhibits protein isoprenylation, depletes plasma glutamine, increases production of foetal haemoglobin through transcriptional activation of the gamma-globin gene and affects hPPARgamma activation.

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 Excerpts

ExcerptRelevanceReference
" Though the treatment was successful in ameliorating hyperammonemia, hyperargininemia persisted."3.96Sodium phenylbutyrate improved the clinical state in an adult patient with arginase 1 deficiency. ( Fukui, K; Hanai, T; Ishibashi, H; Matsufuji, M; Nakashima, M; Takashima, S; Takeshita, E; Watanabe, Y, 2020)

Research

Studies (1)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's1 (100.00)2.80

Authors

AuthorsStudies
Matsufuji, M1
Takeshita, E1
Nakashima, M1
Watanabe, Y1
Fukui, K1
Hanai, T1
Ishibashi, H1
Takashima, S1

Other Studies

1 other study available for 4-phenylbutyric acid and Hyperargininemia

ArticleYear
Sodium phenylbutyrate improved the clinical state in an adult patient with arginase 1 deficiency.
    Brain & development, 2020, Volume: 42, Issue:2

    Topics: Adult; Arginase; Arginine; Female; Humans; Hyperammonemia; Hyperargininemia; Phenylbutyrates

2020