4-phenylbutyric acid has been researched along with Cystic Fibrosis in 17 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.
Cystic Fibrosis: An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.
| Excerpt | Relevance | Reference |
|---|---|---|
| " 4-Phenylbutyrate (Buphenyl), an orally bioavailable short chain fatty acid, modulates heat shock protein expression and restores maturation of the deltaF508 protein in vitro and in vivo." | 2.70 | Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate. ( Boyle, MP; Brass-Ernst, L; Diener-West, M; Lee, CK; Rubenstein, RC; Zeitlin, PL, 2002) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 3 (17.65) | 18.2507 |
| 2000's | 8 (47.06) | 29.6817 |
| 2010's | 6 (35.29) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Chung, WJ | 1 |
| Goeckeler-Fried, JL | 1 |
| Havasi, V | 1 |
| Chiang, A | 1 |
| Rowe, SM | 1 |
| Plyler, ZE | 1 |
| Hong, JS | 1 |
| Mazur, M | 1 |
| Piazza, GA | 1 |
| Keeton, AB | 1 |
| White, EL | 1 |
| Rasmussen, L | 1 |
| Weissman, AM | 1 |
| Denny, RA | 1 |
| Brodsky, JL | 1 |
| Sorscher, EJ | 1 |
| Southern, KW | 1 |
| Patel, S | 1 |
| Sinha, IP | 1 |
| Nevitt, SJ | 1 |
| Molina, SA | 1 |
| Stauffer, B | 1 |
| Moriarty, HK | 1 |
| Kim, AH | 1 |
| McCarty, NA | 1 |
| Koval, M | 1 |
| Suaud, L | 2 |
| Miller, K | 2 |
| Alvey, L | 1 |
| Yan, W | 1 |
| Robay, A | 1 |
| Kebler, C | 1 |
| Kreindler, JL | 1 |
| Guttentag, S | 1 |
| Hubbard, MJ | 1 |
| Rubenstein, RC | 7 |
| Panichelli, AE | 1 |
| Randell, RL | 1 |
| Marando, CM | 1 |
| Nieddu, E | 1 |
| Pollarolo, B | 1 |
| Merello, L | 1 |
| Schenone, S | 1 |
| Mazzei, M | 1 |
| Powell, K | 1 |
| Zeitlin, PL | 8 |
| Lim, M | 1 |
| McKenzie, K | 1 |
| Floyd, AD | 1 |
| Kwon, E | 1 |
| Singh, OV | 1 |
| Vij, N | 1 |
| Mogayzel, PJ | 1 |
| Jozwik, C | 1 |
| Pollard, HB | 1 |
| Prulière-Escabasse, V | 1 |
| Planès, C | 1 |
| Escudier, E | 1 |
| Fanen, P | 1 |
| Coste, A | 1 |
| Clerici, C | 1 |
| Egan, ME | 1 |
| Andersson, C | 1 |
| Roomans, GM | 1 |
| McGrath-Morrow, SA | 1 |
| Stahl, JL | 1 |
| Diener-West, M | 1 |
| Boyle, MP | 1 |
| Lee, CK | 1 |
| Brass-Ernst, L | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Phenylbutyrate for Monogenetic Developmental and Epileptic Encephalopathy[NCT04937062] | Early Phase 1 | 50 participants (Anticipated) | Interventional | 2021-03-01 | Enrolling by invitation | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
4 reviews available for 4-phenylbutyric acid and Cystic Fibrosis
| Article | Year |
|---|---|
Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis.
Topics: Adult; Aminophenols; Aminopyridines; Benzodioxoles; Child; Cystic Fibrosis; Cystic Fibrosis Transmem | 2018 |
F508del-CFTR rescue: a matter of cell stress response.
Topics: Alkaloids; Animals; Cell Membrane; Cold Temperature; Curcumin; Cystic Fibrosis; Cystic Fibrosis Tran | 2013 |
Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting.
Topics: Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Endoplasmic Reticulum | 2002 |
Use of protein repair therapy in the treatment of cystic fibrosis.
Topics: Codon, Terminator; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Gene Expres | 1998 |
2 trials available for 4-phenylbutyric acid and Cystic Fibrosis
| Article | Year |
|---|---|
A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function.
Topics: Administration, Oral; Adolescent; Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane C | 1998 |
Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate.
Topics: Adult; Biological Transport, Active; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Condu | 2002 |
11 other studies available for 4-phenylbutyric acid and Cystic Fibrosis
| Article | Year |
|---|---|
Increasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule Therapeutics.
Topics: Alleles; Benzoates; Cells, Cultured; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regu | 2016 |
Junctional abnormalities in human airway epithelial cells expressing F508del CFTR.
Topics: Adult; Calcium Signaling; Cell Line; Colforsin; Connexin 26; Connexin 43; Connexins; Cystic Fibrosis | 2015 |
ERp29 regulates DeltaF508 and wild-type cystic fibrosis transmembrane conductance regulator (CFTR) trafficking to the plasma membrane in cystic fibrosis (CF) and non-CF epithelial cells.
Topics: Animals; Biotinylation; Cell Membrane; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Re | 2011 |
4-Phenylbutyrate stimulates Hsp70 expression through the Elp2 component of elongator and STAT-3 in cystic fibrosis epithelial cells.
Topics: Antineoplastic Agents; Cell Line, Tumor; Cystic Fibrosis; Epithelial Cells; Gene Expression Regulati | 2011 |
Modulation of deltaF508 cystic fibrosis transmembrane regulator trafficking and function with 4-phenylbutyrate and flavonoids.
Topics: Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relat | 2004 |
Pharmacoproteomics of 4-phenylbutyrate-treated IB3-1 cystic fibrosis bronchial epithelial cells.
Topics: Bronchi; Cell Line, Transformed; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulato | 2006 |
Modulation of epithelial sodium channel trafficking and function by sodium 4-phenylbutyrate in human nasal epithelial cells.
Topics: Antineoplastic Agents; Brefeldin A; Cell Membrane; Cells, Cultured; Cystic Fibrosis; Cystic Fibrosis | 2007 |
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.
Topics: Biological Transport; Cells, Cultured; Chlorides; Colforsin; Cystic Fibrosis; Cystic Fibrosis Transm | 1997 |
Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR.
Topics: Animals; Antineoplastic Agents; Biological Transport; Butyrates; Calcium-Binding Proteins; Calnexin; | 2000 |
Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX.
Topics: Cell Line; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epithelial Cells; G | 2000 |
G(1) Phase growth arrest and induction of p21(Waf1/Cip1/Sdi1) in IB3-1 cells treated with 4-sodium phenylbutyrate.
Topics: Antineoplastic Agents; Apoptosis; Blotting, Western; Cell Division; Cell Line; Cyclin-Dependent Kina | 2000 |