valproic acid has been researched along with Machado-Joseph Disease in 6 studies
Valproic Acid: A fatty acid with anticonvulsant and anti-manic properties that is used in the treatment of EPILEPSY and BIPOLAR DISORDER. The mechanisms of its therapeutic actions are not well understood. It may act by increasing GAMMA-AMINOBUTYRIC ACID levels in the brain or by altering the properties of VOLTAGE-GATED SODIUM CHANNELS.
valproic acid : A branched-chain saturated fatty acid that comprises of a propyl substituent on a pentanoic acid stem.
Machado-Joseph Disease: A dominantly-inherited ATAXIA first described in people of Azorean and Portuguese descent, and subsequently identified in Brazil, Japan, China, and Australia. This disorder is classified as one of the SPINOCEREBELLAR ATAXIAS (Type 3) and has been associated with a mutation of the MJD1 gene on chromosome 14. Clinical features include progressive ataxia, DYSARTHRIA, postural instability, nystagmus, eyelid retraction, and facial FASCICULATIONS. DYSTONIA is prominent in younger patients (referred to as Type I Machado-Joseph Disease). Type II features ataxia and ocular signs; Type III features MUSCULAR ATROPHY and a sensorimotor neuropathy; and Type IV features extrapyramidal signs combined with a sensorimotor neuropathy. (From Clin Neurosci 1995;3(1):17-22; Ann Neurol 1998 Mar;43(3):288-96)
| Excerpt | Relevance | Reference |
|---|---|---|
| " In spite of limited results, the use of another dosage of VPA or of VPA in a combined therapy with molecules targeting other pathways, cannot be excluded as potential strategies for MJD therapeutics." | 5.42 | Limited Effect of Chronic Valproic Acid Treatment in a Mouse Model of Machado-Joseph Disease. ( Duarte-Silva, S; Esteves, S; Maciel, P; Naia, L; Neves-Carvalho, A; Rego, AC; Silva-Fernandes, A; Teixeira-Castro, A, 2015) |
| "Valproic acid (VPA) is a pan-HDAC inhibitor used clinically to treat bipolar and seizure disorders." | 2.82 | Safety and efficacy of valproic acid treatment in SCA3/MJD patients. ( Chuang, DM; Jiang, H; Lei, LF; Song, WH; Tang, BS; Wang, JL; Yang, GP, 2016) |
| "Machado-Joseph disease (MJD, also known as spinocerebellar ataxia type 3) is a fatal neurodegenerative disease that impairs control and coordination of movement." | 1.62 | Sodium valproate increases activity of the sirtuin pathway resulting in beneficial effects for spinocerebellar ataxia-3 in vivo. ( Chung, RS; Cole, NJ; De Luca, A; Guillemin, GJ; Laird, AS; Lee, A; Luu, L; Nicholson, GA; Robinson, KJ; Suddull, HJ; Tym, MC; Watchon, M; Yuan, KC, 2021) |
| " In spite of limited results, the use of another dosage of VPA or of VPA in a combined therapy with molecules targeting other pathways, cannot be excluded as potential strategies for MJD therapeutics." | 1.42 | Limited Effect of Chronic Valproic Acid Treatment in a Mouse Model of Machado-Joseph Disease. ( Duarte-Silva, S; Esteves, S; Maciel, P; Naia, L; Neves-Carvalho, A; Rego, AC; Silva-Fernandes, A; Teixeira-Castro, A, 2015) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 1 (16.67) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 4 (66.67) | 24.3611 |
| 2020's | 1 (16.67) | 2.80 |
| Authors | Studies |
|---|---|
| Watchon, M | 1 |
| Luu, L | 1 |
| Robinson, KJ | 1 |
| Yuan, KC | 1 |
| De Luca, A | 1 |
| Suddull, HJ | 1 |
| Tym, MC | 1 |
| Guillemin, GJ | 1 |
| Cole, NJ | 1 |
| Nicholson, GA | 1 |
| Chung, RS | 1 |
| Lee, A | 1 |
| Laird, AS | 1 |
| Wang, Z | 1 |
| He, F | 1 |
| Abeditashi, M | 1 |
| Schmidt, T | 1 |
| Esteves, S | 1 |
| Duarte-Silva, S | 1 |
| Naia, L | 1 |
| Neves-Carvalho, A | 1 |
| Teixeira-Castro, A | 1 |
| Rego, AC | 1 |
| Silva-Fernandes, A | 1 |
| Maciel, P | 1 |
| Lei, LF | 1 |
| Yang, GP | 1 |
| Wang, JL | 1 |
| Chuang, DM | 1 |
| Song, WH | 1 |
| Tang, BS | 1 |
| Jiang, H | 2 |
| Yi, J | 1 |
| Zhang, L | 1 |
| Tang, B | 1 |
| Han, W | 1 |
| Zhou, Y | 1 |
| Chen, Z | 1 |
| Jia, D | 1 |
| Ellaway, CJ | 1 |
| Bennetts, B | 1 |
| Tuck, RR | 1 |
| Wilcken, B | 1 |
1 trial available for valproic acid and Machado-Joseph Disease
| Article | Year |
|---|---|
Safety and efficacy of valproic acid treatment in SCA3/MJD patients.
Topics: Adult; Dose-Response Relationship, Drug; Double-Blind Method; Enzyme Inhibitors; Female; Histone Dea | 2016 |
5 other studies available for valproic acid and Machado-Joseph Disease
| Article | Year |
|---|---|
Sodium valproate increases activity of the sirtuin pathway resulting in beneficial effects for spinocerebellar ataxia-3 in vivo.
Topics: Acetylation; Animals; Animals, Genetically Modified; Ataxin-3; Autophagy; Carbazoles; Disease Models | 2021 |
Divalproex sodium regulates ataxin-3 translocation likely by an importin α1-dependent pathway.
Topics: alpha Karyopherins; Animals; Ataxin-3; Cell Nucleus; CHO Cells; Cricetulus; Gene Expression; HEK293 | 2019 |
Limited Effect of Chronic Valproic Acid Treatment in a Mouse Model of Machado-Joseph Disease.
Topics: Animals; Astrocytes; Ataxin-3; Brain; Disease Models, Animal; Endoplasmic Reticulum Chaperone BiP; H | 2015 |
Sodium valproate alleviates neurodegeneration in SCA3/MJD via suppressing apoptosis and rescuing the hypoacetylation levels of histone H3 and H4.
Topics: Acetylation; Animals; Animals, Genetically Modified; Apoptosis; Cells, Cultured; DNA Repeat Expansio | 2013 |
Clumsiness, confusion, coma, and valproate.
Topics: Adult; Coma; Confusion; Fatal Outcome; GABA Agents; Humans; Machado-Joseph Disease; Male; Valproic A | 1999 |