4-phenylbutyric acid has been researched along with Hepatolenticular Degeneration 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.
Hepatolenticular Degeneration: A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 1 (50.00) | 29.6817 |
| 2010's | 1 (50.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Kumari, N | 1 |
| Kumar, A | 1 |
| Thapa, BR | 1 |
| Modi, M | 1 |
| Pal, A | 1 |
| Prasad, R | 1 |
| van den Berghe, PV | 1 |
| Stapelbroek, JM | 1 |
| Krieger, E | 1 |
| de Bie, P | 1 |
| van de Graaf, SF | 1 |
| de Groot, RE | 1 |
| van Beurden, E | 1 |
| Spijker, E | 1 |
| Houwen, RH | 1 |
| Berger, R | 1 |
| Klomp, LW | 1 |
2 other studies available for 4-phenylbutyric acid and Hepatolenticular Degeneration
| Article | Year |
|---|---|
Characterization of mutation spectrum and identification of novel mutations in ATP7B gene from a cohort of Wilson disease patients: Functional and therapeutic implications.
Topics: Adolescent; Adult; Case-Control Studies; Cell Line; Cell Survival; Child; Child, Preschool; Cohort S | 2018 |
Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin.
Topics: Adaptor Proteins, Signal Transducing; Adenosine Triphosphatases; Carrier Proteins; Cation Transport | 2009 |