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4-phenylbutyric acid and Hyperammonemia

4-phenylbutyric acid has been researched along with Hyperammonemia in 13 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.

Hyperammonemia: Elevated level of AMMONIA in the blood. It is a sign of defective CATABOLISM of AMINO ACIDS or ammonia to UREA.

Research Excerpts

ExcerptRelevanceReference
"Hyperammonemia has been reported following asparaginase administration, consistent with the mechanisms of asparaginase, which catabolizes asparagine to aspartic acid and ammonia, and secondarily converts glutamine to glutamate and ammonia."4.31Treatment and outcomes of symptomatic hyperammonemia following asparaginase therapy in children with acute lymphoblastic leukemia. ( Altintas, B; Eldem, I; Langley, R; Lee, A; Nguyen, H; Shinawi, M; Willis, D, 2023)
" 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)
" Her hyperammonemia was corrected by hemodialysis, followed by sodium benzoate, L-arginine, levocarnitine and protein-free diet therapy."3.96Long-term survival of a patient with acute neonatal-onset metabolic encephalopathy with carbamoyl phosphate synthetase 1 deficiency. ( Ando, Y; Arisaka, O; Imataka, G; Ishii, J; Nitta, A; Takagi, Y; Yoshihara, S, 2020)
" Four Phase 1 studies were conducted to characterize the bioavailability (BA) and/or bioequivalence (BE) of ACER-001 (in healthy volunteers) and taste assessment relative to NaPBA powder (in taste panelists)."1.91Taste-masked formulation of sodium phenylbutyrate (ACER-001) for the treatment of urea cycle disorders. ( Cederbaum, SD; Edwards, J; Kellmeyer, T; Peters, Y; Steiner, RD, 2023)

Research

Studies (13)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's6 (46.15)24.3611
2020's7 (53.85)2.80

Authors

AuthorsStudies
Cederbaum, SD1
Edwards, J1
Kellmeyer, T1
Peters, Y1
Steiner, RD1
Eriksen, PL1
Djernes, L1
Vilstrup, H1
Ott, P1
Lee, A1
Eldem, I1
Altintas, B1
Nguyen, H1
Willis, D1
Langley, R1
Shinawi, M1
Mangum, R1
Soler-Alfonso, C1
Birchansky, S1
Stevens, A1
Matsufuji, M1
Takeshita, E1
Nakashima, M1
Watanabe, Y1
Fukui, K1
Hanai, T1
Ishibashi, H1
Takashima, S1
Imataka, G1
Ishii, J1
Ando, Y1
Yoshihara, S2
Takagi, Y1
Nitta, A1
Arisaka, O1
Sato, T1
Ishii, T1
Kato, M1
Oyanagi, T1
Nakatsuka, S1
Kuroda, T1
Hasegawa, T1
Weiss, N1
Tripon, S1
Lodey, M1
Guiller, E1
Junot, H1
Monneret, D1
Mayaux, J1
Brisson, H1
Mallet, M1
Rudler, M1
Imbert-Bismut, F1
Thabut, D1
Matson, KM1
Sonetti, DA1
Giva, S1
Finnegan, J1
Ihidero, P1
Maguire, G1
Power, B1
Knerr, I1
Monavari, A1
van Straten, G1
van Dalen, D1
Mesu, SJ1
Rothuizen, J1
Teske, E1
Spee, B1
Favier, RP1
van Geijlswijk, IM1
Bender, HU1
Staudigl, M1
Schmid, I1
Führer, M1
Nagamani, SC1
Erez, A1
Lee, B1

Reviews

1 review available for 4-phenylbutyric acid and Hyperammonemia

ArticleYear
Argininosuccinate lyase deficiency.
    Genetics in medicine : official journal of the American College of Medical Genetics, 2012, Volume: 14, Issue:5

    Topics: Arginine; Argininosuccinate Lyase; Argininosuccinic Acid; Argininosuccinic Aciduria; Child, Preschoo

2012

Trials

2 trials available for 4-phenylbutyric acid and Hyperammonemia

ArticleYear
Clearance and production of ammonia quantified in humans by constant ammonia infusion - the effects of cirrhosis and ammonia-targeting treatments.
    Journal of hepatology, 2023, Volume: 79, Issue:2

    Topics: Ammonia; Hepatic Encephalopathy; Humans; Hyperammonemia; Lactulose; Liver Cirrhosis; Male; Phenylbut

2023
Efficacy of orally administered sodium benzoate and sodium phenylbutyrate in dogs with congenital portosystemic shunts.
    Journal of veterinary internal medicine, 2019, Volume: 33, Issue:3

    Topics: Ammonia; Animals; Bile Acids and Salts; Cross-Over Studies; Dogs; Double-Blind Method; Female; Hyper

2019

Other Studies

10 other studies available for 4-phenylbutyric acid and Hyperammonemia

ArticleYear
Taste-masked formulation of sodium phenylbutyrate (ACER-001) for the treatment of urea cycle disorders.
    Molecular genetics and metabolism, 2023, Volume: 138, Issue:4

    Topics: Humans; Hyperammonemia; Nitrogen; Phenylbutyrates; Powders; Rare Diseases; Taste; Urea; Urea Cycle D

2023
Treatment and outcomes of symptomatic hyperammonemia following asparaginase therapy in children with acute lymphoblastic leukemia.
    Molecular genetics and metabolism, 2023, Volume: 139, Issue:3

    Topics: Ammonia; Antineoplastic Agents; Asparaginase; Child; Glutamine; Humans; Hyperammonemia; Phenylbutyra

2023
Survival and Neurologic Recovery After Prompt Diagnosis and Aggressive Management of Severe Idiopathic Hyperammonemic Encephalopathy in a Patient with Acute Myeloid Leukemia.
    Journal of pediatric hematology/oncology, 2020, Volume: 42, Issue:6

    Topics: Adult; Brain Diseases; Female; Humans; Hyperammonemia; Leukemia, Myeloid, Acute; Neurotoxicity Syndr

2020
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
Long-term survival of a patient with acute neonatal-onset metabolic encephalopathy with carbamoyl phosphate synthetase 1 deficiency.
    European review for medical and pharmacological sciences, 2020, Volume: 24, Issue:19

    Topics: Arginine; Brain Diseases, Metabolic; Carbamoyl-Phosphate Synthase I Deficiency Disease; Carnitine; F

2020
Oral sodium phenylbutyrate for hyperammonemia associated with congenital portosystemic shunt: a case report.
    Journal of pediatric endocrinology & metabolism : JPEM, 2021, Mar-26, Volume: 34, Issue:3

    Topics: Administration, Oral; Amino Acids, Branched-Chain; Ammonia; Humans; Hyperammonemia; Infant; Male; Ph

2021
Treating hepatic encephalopathy in cirrhotic patients admitted to ICU with sodium phenylbutyrate: a preliminary study.
    Fundamental & clinical pharmacology, 2018, Volume: 32, Issue:2

    Topics: Aged; Ammonia; Biomarkers; Case-Control Studies; Female; Hepatic Encephalopathy; Hospital Mortality;

2018
Successful treatment of Ureaplasma-induced hyperammonemia syndrome post-lung transplant.
    Transplant infectious disease : an official journal of the Transplantation Society, 2019, Volume: 21, Issue:1

    Topics: Anti-Infective Agents; Bronchoalveolar Lavage Fluid; Graft Rejection; Humans; Hyperammonemia; Immuno

2019
Hyperammonaemia in Neonates and Young Children: Potential Metabolic Causes, Diagnostic Approaches and Clinical Consequences
    Irish medical journal, 2019, 01-15, Volume: 112, Issue:1

    Topics: Ammonia; Arginine; Biomarkers; Brain Diseases, Metabolic, Inborn; Carnitine; Critical Illness; Diet,

2019
Treatment of Paraneoplastic Hyperammonemia in Fibrolamellar Hepatocellular Carcinoma With Oral Sodium Phenylbutyrate.
    Journal of pain and symptom management, 2015, Volume: 49, Issue:6

    Topics: Administration, Oral; Antineoplastic Agents; Carcinoma, Hepatocellular; Fatal Outcome; Female; Human

2015