4-phenylbutyric acid and glycerol

4-phenylbutyric acid has been researched along with glycerol in 17 studies

Research

Studies (17)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's6 (35.29)29.6817
2010's11 (64.71)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Rubenstein, RC; Zeitlin, PL1
Burrows, JA; Perlmutter, DH; Willis, LK1
Csermely, P; Papp, E1
Benos, DJ; Bubien, JK; Colby, G; Esimai, O; Fuller, CM; Gillespie, GY; Jovov, B; Kovacs, GG; Mapstone, TB; Markert, JM; Pahwa, AK; Vila-Carriles, WH; Zhou, ZH1
Crilley, TK; Morrell, NW; Rudarakanchana, N; Sobolewski, A; Trembath, RC; Upton, PD; Yang, J1
Leandro, J; Leandro, P; Nascimento, C; Tavares de Almeida, I1
Beliveau, M; Berry, SA; Diaz, GA; Dickinson, K; Gargosky, S; Lee, B; Marier, JF; Martinez, A; Mauney, J; Mian, A; Mokhtarani, M; Rhead, W; Scharschmidt, BF; Shchelochkov, O1
Beliveau, M; Diaz, GA; Dickinson, K; Feigenbaum, A; Jomphe, C; Lichter-Konecki, U; Marier, JF; Martinez, A; Mauney, J; Merritt, JL; Mokhtarani, M; Rhead, W; Scharschmidt, B1
Aït-Slimane, T; Delaunay, JL; Delautier, D; Durand-Schneider, AM; Gabillet, J; Gautherot, J; Housset, C; Maurice, M; Rada, A1
Bart, SA; Bartholomew, D; Bartley, J; Berquist, W; Berry, SA; C S Nagamani, S; Cederbaum, S; Coakley, DF; Diaz, GA; Dickinson, K; Feigenbaum, A; Gallagher, R; Harding, CO; Korson, MS; Krivitzky, LS; Kronn, D; Lee, B; Lemons, C; Lichter-Konecki, U; Longo, N; Mauney, J; McCandless, SE; Merritt, JL; Mokhtarani, M; Moors, TL; Rhead, W; Scharschmidt, BF; Smith, W; Vockley, G; Zori, R1
Mistry, PK1
Berry, SA; Coakley, DF; Diaz, GA; Dickinson, K; Harding, CO; Lee, B; LeMons, C; Lichter-Konecki, U; Mauney, J; McCandless, SE; Mokhtarani, M; Moors, T; Scharschmidt, BF; Smith, W1
Guha, M1
Agrawal, A; Chakraborty, K; Chakraborty, S; Ghosh, B; Krishnan, V; Mabalirajan, U; Maity, S; Makhija, L; Rehman, R1
Chen, YL; Ding, XY; Hou, FF; Hu, LL; Li, C; Xu, GY; Zhou, QG; Zhu, Y1
Bok, D; Hu, J; Izumi, T; Jacobson, SG; Jin, HH; Jin, M; Li, S; Siddiqui, AA1
Carlisle, RE; Dickhout, JG; Upagupta, C1

Trials

4 trial(s) available for 4-phenylbutyric acid and glycerol

ArticleYear
Phase 2 comparison of a novel ammonia scavenging agent with sodium phenylbutyrate in patients with urea cycle disorders: safety, pharmacokinetics and ammonia control.
    Molecular genetics and metabolism, 2010, Volume: 100, Issue:3

    Topics: Adult; Aged; Ammonia; Cross-Over Studies; Female; Glutamine; Glycerol; Humans; Male; Middle Aged; Phenylacetates; Phenylbutyrates; Urea Cycle Disorders, Inborn; Young Adult

2010
Ammonia control in children with urea cycle disorders (UCDs); phase 2 comparison of sodium phenylbutyrate and glycerol phenylbutyrate.
    Molecular genetics and metabolism, 2011, Volume: 103, Issue:4

    Topics: Adolescent; Ammonia; Child; Dose-Response Relationship, Drug; Glycerol; Humans; Male; Phenylbutyrates; Urea; Urea Cycle Disorders, Inborn

2011
Ammonia control and neurocognitive outcome among urea cycle disorder patients treated with glycerol phenylbutyrate.
    Hepatology (Baltimore, Md.), 2013, Volume: 57, Issue:6

    Topics: Adolescent; Adult; Ammonia; Child; Cross-Over Studies; Double-Blind Method; Female; Glutamine; Glycerol; Humans; Male; Middle Aged; Neuropsychological Tests; Phenylbutyrates; Urea Cycle Disorders, Inborn; Young Adult

2013
Ammonia control in children ages 2 months through 5 years with urea cycle disorders: comparison of sodium phenylbutyrate and glycerol phenylbutyrate.
    The Journal of pediatrics, 2013, Volume: 162, Issue:6

    Topics: Ammonia; Child; Child, Preschool; Drug Substitution; Female; Glutamine; Glycerol; Humans; Infant; Liver; Male; Phenylbutyrates; Surveys and Questionnaires; Urea Cycle Disorders, Inborn

2013

Other Studies

13 other study(ies) available for 4-phenylbutyric acid and glycerol

ArticleYear
Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR.
    American journal of physiology. Cell physiology, 2000, Volume: 278, Issue:2

    Topics: Animals; Antineoplastic Agents; Biological Transport; Butyrates; Calcium-Binding Proteins; Calnexin; Carrier Proteins; Cells, Cultured; Cysteine Endopeptidases; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Down-Regulation; Gene Expression; Glycerol; Heat-Shock Proteins; HSC70 Heat-Shock Proteins; HSP40 Heat-Shock Proteins; HSP70 Heat-Shock Proteins; HSP90 Heat-Shock Proteins; Humans; Multienzyme Complexes; Mutation; Phenylbutyrates; Proteasome Endopeptidase Complex; Rabbits; RNA, Messenger; Ubiquitins

2000
Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency.
    Proceedings of the National Academy of Sciences of the United States of America, 2000, Feb-15, Volume: 97, Issue:4

    Topics: Administration, Oral; alpha 1-Antitrypsin; alpha 1-Antitrypsin Deficiency; Animals; Cell Line; Emphysema; Glycerol; Humans; Liver; Lung; Methylamines; Mice; Mice, Transgenic; Mutation; Phenylbutyrates; Protein Folding; Temperature

2000
Chemical chaperones: mechanisms of action and potential use.
    Handbook of experimental pharmacology, 2006, Issue:172

    Topics: Animals; Enzyme Inhibitors; Glycerol; Humans; Methylamines; Molecular Chaperones; Phenylbutyrates; Protein Folding

2006
Surface expression of ASIC2 inhibits the amiloride-sensitive current and migration of glioma cells.
    The Journal of biological chemistry, 2006, Jul-14, Volume: 281, Issue:28

    Topics: Acid Sensing Ion Channels; Amiloride; Antineoplastic Agents; Brain Neoplasms; Cell Membrane; Cell Movement; Cell Proliferation; Glioblastoma; Glioma; Glycerol; Humans; Membrane Proteins; Nerve Tissue Proteins; Phenylbutyrates; Sodium; Sodium Channel Blockers; Sodium Channels

2006
Failure of bone morphogenetic protein receptor trafficking in pulmonary arterial hypertension: potential for rescue.
    Human molecular genetics, 2008, Oct-15, Volume: 17, Issue:20

    Topics: Amino Acid Substitution; Biological Transport, Active; Bone Morphogenetic Protein Receptors, Type I; Bone Morphogenetic Protein Receptors, Type II; Cell Membrane; Endoplasmic Reticulum; Germ-Line Mutation; Glycerol; HeLa Cells; Humans; Hypertension, Pulmonary; Models, Biological; Phenylbutyrates; Recombinant Proteins; Signal Transduction; Smad Proteins, Receptor-Regulated; Thapsigargin; Transfection

2008
Modulation of the activity of newly synthesized human phenylalanine hydroxylase mutant proteins by low-molecular-weight compounds.
    The protein journal, 2008, Volume: 27, Issue:6

    Topics: Animals; Escherichia coli; Escherichia coli Proteins; Gene Expression Regulation, Enzymologic; Glycerol; Humans; Methylamines; Mutant Proteins; Phenylalanine Hydroxylase; Phenylbutyrates; Phenylketonurias; Recombinant Fusion Proteins; Temperature

2008
Effects of cellular, chemical, and pharmacological chaperones on the rescue of a trafficking-defective mutant of the ATP-binding cassette transporter proteins ABCB1/ABCB4.
    The Journal of biological chemistry, 2012, Feb-10, Volume: 287, Issue:7

    Topics: Amino Acid Substitution; Animals; Antineoplastic Agents; ATP Binding Cassette Transporter, Subfamily B; ATP Binding Cassette Transporter, Subfamily B, Member 1; Calnexin; Cholestasis; Cryoprotective Agents; Cyclosporine; Dogs; Endoplasmic Reticulum; Enzyme Inhibitors; Gene Silencing; Glycerol; Hep G2 Cells; HSC70 Heat-Shock Proteins; Humans; Mutation, Missense; Phenylbutyrates; Protein Transport; Thapsigargin

2012
Rare disease clinical research network's urea cycle consortium delivers a successful clinical trial to improve alternate pathway therapy.
    Hepatology (Baltimore, Md.), 2013, Volume: 57, Issue:6

    Topics: Ammonia; Female; Glycerol; Humans; Male; Phenylbutyrates; Urea Cycle Disorders, Inborn

2013
Urea cycle disorder drug approved.
    Nature biotechnology, 2013, Volume: 31, Issue:4

    Topics: Adult; Clinical Trials as Topic; Drug Approval; Drug Industry; Glycerol; Humans; Patient Compliance; Phenylbutyrates; Urea Cycle Disorders, Inborn

2013
Chemical chaperones mitigate experimental asthma by attenuating endoplasmic reticulum stress.
    American journal of respiratory cell and molecular biology, 2014, Volume: 50, Issue:5

    Topics: Airway Remodeling; Animals; Asthma; Endoplasmic Reticulum; Endoplasmic Reticulum Stress; Glycerol; Inflammation; Lung; Male; Methylamines; Mice; Mice, Inbred BALB C; Molecular Chaperones; Phenylbutyrates; Protein Folding; Trehalose; Unfolded Protein Response

2014
The effect of inhibition of endoplasmic reticulum stress on lipolysis in white adipose tissue in a rat model of chronic kidney disease.
    Acta pharmacologica Sinica, 2014, Volume: 35, Issue:3

    Topics: Acyltransferases; Adipocytes; Animals; Cells, Cultured; Disease Models, Animal; eIF-2 Kinase; Endoplasmic Reticulum; Endoplasmic Reticulum Stress; Eukaryotic Initiation Factor-2; Glycerol; Intra-Abdominal Fat; Lipase; Lipolysis; Male; Membrane Proteins; Phenylbutyrates; Phosphorylation; Protein Serine-Threonine Kinases; Rats, Sprague-Dawley; Renal Insufficiency, Chronic; Signal Transduction; Sterol Esterase

2014
Rescue of enzymatic function for disease-associated RPE65 proteins containing various missense mutations in non-active sites.
    The Journal of biological chemistry, 2014, Jul-04, Volume: 289, Issue:27

    Topics: Animals; Catalytic Domain; Cell Membrane; cis-trans-Isomerases; Disease; Gene Expression Regulation, Enzymologic; Glycerol; HEK293 Cells; Humans; Mice; Models, Molecular; Mutant Proteins; Mutation, Missense; Phenylbutyrates; Proteasome Endopeptidase Complex; Protein Folding; Proteolysis; Retinal Pigment Epithelium; Temperature; Ubiquitination

2014
Analysis of the potency of various low molecular weight chemical chaperones to prevent protein aggregation.
    Biochemical and biophysical research communications, 2017, 04-22, Volume: 486, Issue:1

    Topics: Benzothiazoles; Cell Line; Docosahexaenoic Acids; Endoplasmic Reticulum Stress; Epithelial Cells; Glycerol; Humans; Kidney Tubules, Proximal; Molecular Weight; Phenylbutyrates; Protein Aggregates; Protein Aggregation, Pathological; Protein Folding; Staining and Labeling; Taurochenodeoxycholic Acid; Thapsigargin; Thiazoles; Trehalose; Unfolded Protein Response; Xenobiotics

2017