4-phenylbutyric acid has been researched along with Wolfram Syndrome in 2 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.
Wolfram Syndrome: A hereditary condition characterized by multiple symptoms including those of DIABETES INSIPIDUS; DIABETES MELLITUS; OPTIC ATROPHY; and DEAFNESS. This syndrome is also known as DIDMOAD (first letter of each word) and is usually associated with VASOPRESSIN deficiency. It is caused by mutations in gene WFS1 encoding wolframin, a 100-kDa transmembrane protein.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Wolfram syndrome is an autosomal recessive disorder caused by mutations in WFS1 and is characterized by insulin-dependent diabetes mellitus, optic atrophy, and deafness." | 1.40 | β-cell dysfunction due to increased ER stress in a stem cell model of Wolfram syndrome. ( Chung, W; Egli, D; Foo, K; Freeby, M; Goland, R; Hua, H; Kahler, DJ; LeDuc, C; Leibel, RL; Martinez, H; Shang, L; Watanabe, K; Zimmer, M, 2014) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 1 (50.00) | 24.3611 |
| 2020's | 1 (50.00) | 2.80 |
| Authors | Studies |
|---|---|
| Batjargal, K | 1 |
| Tajima, T | 1 |
| Jimbo, EF | 1 |
| Yamagata, T | 1 |
| Shang, L | 1 |
| Hua, H | 1 |
| Foo, K | 1 |
| Martinez, H | 1 |
| Watanabe, K | 1 |
| Zimmer, M | 1 |
| Kahler, DJ | 1 |
| Freeby, M | 1 |
| Chung, W | 1 |
| LeDuc, C | 1 |
| Goland, R | 1 |
| Leibel, RL | 1 |
| Egli, D | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Treatment of Wolfram Syndrome Type 2 With the Chelator Deferiprone, and Incretin Based Therapy[NCT02882477] | Phase 2/Phase 3 | 20 participants (Anticipated) | Interventional | 2016-12-31 | Not yet recruiting | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
2 other studies available for 4-phenylbutyric acid and Wolfram Syndrome
| Article | Year |
|---|---|
Effect of 4-phenylbutyrate and valproate on dominant mutations of WFS1 gene in Wolfram syndrome.
Topics: Animals; Apoptosis; Cells, Cultured; Endoplasmic Reticulum Stress; Gene Expression Regulation; Genes | 2020 |
β-cell dysfunction due to increased ER stress in a stem cell model of Wolfram syndrome.
Topics: Animals; Calcium; Cell Differentiation; Endoplasmic Reticulum Stress; Humans; Induced Pluripotent St | 2014 |