4-phenylbutyric acid has been researched along with Adrenoleukodystrophy in 9 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.
Adrenoleukodystrophy: An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS).
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 2 (22.22) | 18.2507 |
| 2000's | 4 (44.44) | 29.6817 |
| 2010's | 2 (22.22) | 24.3611 |
| 2020's | 1 (11.11) | 2.80 |
| Authors | Studies |
|---|---|
| Nemeth, CL | 1 |
| Gӧk, Ö | 1 |
| Tomlinson, SN | 1 |
| Sharma, A | 1 |
| Moser, AB | 2 |
| Kannan, S | 1 |
| Kannan, RM | 1 |
| Fatemi, A | 1 |
| Jang, J | 1 |
| Kang, HC | 1 |
| Kim, HS | 1 |
| Kim, JY | 1 |
| Huh, YJ | 1 |
| Kim, DS | 1 |
| Yoo, JE | 1 |
| Lee, JA | 1 |
| Lim, B | 1 |
| Lee, J | 1 |
| Yoon, TM | 1 |
| Park, IH | 1 |
| Hwang, DY | 1 |
| Daley, GQ | 1 |
| Kim, DW | 1 |
| Brose, RD | 1 |
| Shin, G | 1 |
| McGuinness, MC | 2 |
| Schneidereith, T | 1 |
| Purvis, S | 1 |
| Dong, GX | 1 |
| Keefer, J | 1 |
| Spencer, F | 1 |
| Smith, KD | 2 |
| Imamura, A | 1 |
| Suzuki, Y | 1 |
| Gondcaille, C | 1 |
| Depreter, M | 1 |
| Fourcade, S | 1 |
| Lecca, MR | 1 |
| Leclercq, S | 1 |
| Martin, PG | 1 |
| Pineau, T | 1 |
| Cadepond, F | 1 |
| ElEtr, M | 1 |
| Bertrand, N | 1 |
| Beley, A | 1 |
| Duclos, S | 1 |
| De Craemer, D | 1 |
| Roels, F | 1 |
| Savary, S | 1 |
| Bugaut, M | 1 |
| Kemp, S | 2 |
| Wanders, RJ | 2 |
| Wei, HM | 1 |
| Lu, JF | 1 |
| Braiterman, LT | 1 |
| Watkins, PA | 1 |
| Girard, S | 1 |
| Bruckert, E | 1 |
| Turpin, G | 1 |
3 reviews available for 4-phenylbutyric acid and Adrenoleukodystrophy
| Article | Year |
|---|---|
[X-linked adrenoleukodystrophy (ALD)].
Topics: Adrenoleukodystrophy; Age of Onset; ATP Binding Cassette Transporter, Subfamily D, Member 1; ATP-Bin | 2004 |
X-linked adrenoleukodystrophy: very long-chain fatty acid metabolism, ABC half-transporters and the complicated route to treatment.
Topics: Adrenoleukodystrophy; Animals; ATP Binding Cassette Transporter, Subfamily D; ATP Binding Cassette T | 2007 |
[Endocrine disease in adrenoleukodystrophy].
Topics: Adrenal Insufficiency; Adrenoleukodystrophy; Adult; Anticholesteremic Agents; Bone Marrow Transplant | 2001 |
6 other studies available for 4-phenylbutyric acid and Adrenoleukodystrophy
| Article | Year |
|---|---|
Targeted Brain Delivery of Dendrimer-4-Phenylbutyrate Ameliorates Neurological Deficits in a Long-Term ABCD1-Deficient Mouse Model of X-Linked Adrenoleukodystrophy.
Topics: Adrenoleukodystrophy; Animals; ATP Binding Cassette Transporter, Subfamily D, Member 1; ATP-Binding | 2023 |
Induced pluripotent stem cell models from X-linked adrenoleukodystrophy patients.
Topics: Adrenoleukodystrophy; ATP Binding Cassette Transporter, Subfamily D; ATP-Binding Cassette Transporte | 2011 |
Activation of the stress proteome as a mechanism for small molecule therapeutics.
Topics: Adrenoleukodystrophy; Cell Line; Drug Therapy; Humans; Hydroxamic Acids; Hydroxyurea; Isothiocyanate | 2012 |
Phenylbutyrate up-regulates the adrenoleukodystrophy-related gene as a nonclassical peroxisome proliferator.
Topics: Adrenoleukodystrophy; Animals; ATP Binding Cassette Transporter, Subfamily D; ATP Binding Cassette T | 2005 |
A happier sequel to Lorenzo's Oil?
Topics: Adrenoleukodystrophy; ATP Binding Cassette Transporter, Subfamily D; ATP Binding Cassette Transporte | 1998 |
Gene redundancy and pharmacological gene therapy: implications for X-linked adrenoleukodystrophy.
Topics: Adrenoleukodystrophy; Animals; ATP Binding Cassette Transporter, Subfamily D; ATP Binding Cassette T | 1998 |