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4-phenylbutyric acid and Ornithine Carbamoyltransferase Deficiency Disease

4-phenylbutyric acid has been researched along with Ornithine Carbamoyltransferase Deficiency Disease in 3 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.

Ornithine Carbamoyltransferase Deficiency Disease: An inherited urea cycle disorder associated with deficiency of the enzyme ORNITHINE CARBAMOYLTRANSFERASE, transmitted as an X-linked trait and featuring elevations of amino acids and ammonia in the serum. Clinical features, which are more prominent in males, include seizures, behavioral alterations, episodic vomiting, lethargy, and coma. (Menkes, Textbook of Child Neurology, 5th ed, pp49-50)

Research

Studies (3)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's1 (33.33)18.2507
2000's0 (0.00)29.6817
2010's1 (33.33)24.3611
2020's1 (33.33)2.80

Authors

AuthorsStudies
Zhou, D1
Shang, X1
Qiao, Y1
Cheng, Y1
Yu, Z1
Huang, X1
Hayashi, H1
Mizuno, T1
Horikawa, R1
Nagasaka, H1
Yabuki, T1
Takikawa, H1
Sugiyama, Y1

Reviews

1 review available for 4-phenylbutyric acid and Ornithine Carbamoyltransferase Deficiency Disease

ArticleYear
[Preliminary study of glyceryl phenylbutyrate therapy for Ornithine transcarbamylase deficiency and a literature review].
    Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2023, Sep-10, Volume: 40, Issue:9

    Topics: Ammonia; Child; Humans; Ornithine Carbamoyltransferase Deficiency Disease; Phenylbutyrates; Retrospe

2023

Other Studies

2 other studies available for 4-phenylbutyric acid and Ornithine Carbamoyltransferase Deficiency Disease

ArticleYear
4-Phenylbutyrate modulates ubiquitination of hepatocanalicular MRP2 and reduces serum total bilirubin concentration.
    Journal of hepatology, 2012, Volume: 56, Issue:5

    Topics: Adult; Animals; Bilirubin; Carcinoma, Hepatocellular; Cell Line; Cell Membrane; Child, Preschool; Do

2012
Sodium phenylbutyrate for urea cycle enzyme deficiencies.
    The Medical letter on drugs and therapeutics, 1996, Nov-22, Volume: 38, Issue:988

    Topics: Adult; Amino Acid Metabolism, Inborn Errors; Costs and Cost Analysis; Female; Humans; Infant; Liver;

1996