valproic acid has been researched along with Spinal Muscular Atrophies of Childhood in 12 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.
Spinal Muscular Atrophies of Childhood: A group of recessive inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3)
| Excerpt | Relevance | Reference |
|---|---|---|
| "Hydroxyurea may enhance splice function and increase the number of nuclear 'gems', small nuclear organelles in which survival motor neuron protein concentrates." | 2.44 | Clinical trials in spinal muscular atrophy. ( Darras, BT; Kang, PB, 2007) |
| "Valproic acid may increase SMN levels both by activating the SMN promoter and by preventing exon 7 skipping in SMN transcripts." | 1.32 | Valproic acid increases SMN levels in spinal muscular atrophy patient cells. ( Burghes, AH; Chen, X; Coovert, DD; Fischbeck, KH; Hill, B; Huynh, TN; Jarecki, J; Markowitz, JA; Perhac, JS; Schussler, K; Sumner, CJ; Taylor, JP, 2003) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 4 (33.33) | 29.6817 |
| 2010's | 8 (66.67) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Krosschell, KJ | 2 |
| Kissel, JT | 2 |
| Townsend, EL | 1 |
| Simeone, SD | 1 |
| Zhang, RZ | 1 |
| Reyna, SP | 2 |
| Crawford, TO | 2 |
| Schroth, MK | 2 |
| Acsadi, G | 2 |
| Kishnani, PS | 1 |
| Von Kleist-Retzow, JC | 1 |
| Hero, B | 1 |
| D'Anjou, G | 2 |
| Smith, EC | 1 |
| Elsheikh, B | 2 |
| Simard, LR | 1 |
| Prior, TW | 1 |
| Scott, CB | 1 |
| Lasalle, B | 1 |
| Sakonju, A | 2 |
| Wirth, B | 2 |
| Swoboda, KJ | 2 |
| Yoshida, M | 1 |
| Kitaoka, S | 1 |
| Egawa, N | 1 |
| Yamane, M | 1 |
| Ikeda, R | 1 |
| Tsukita, K | 1 |
| Amano, N | 1 |
| Watanabe, A | 1 |
| Morimoto, M | 1 |
| Takahashi, J | 1 |
| Hosoi, H | 1 |
| Nakahata, T | 1 |
| Inoue, H | 1 |
| Saito, MK | 1 |
| Rak, K | 1 |
| Lechner, BD | 1 |
| Schneider, C | 1 |
| Drexl, H | 1 |
| Sendtner, M | 1 |
| Jablonka, S | 1 |
| Rudnik-Schöneborn, S | 1 |
| Berg, C | 1 |
| Zerres, K | 1 |
| Betzler, C | 1 |
| Grimm, T | 1 |
| Eggermann, T | 1 |
| Eggermann, K | 1 |
| Wirth, R | 1 |
| Heller, R | 1 |
| Piepers, S | 1 |
| Cobben, JM | 1 |
| Sodaar, P | 1 |
| Jansen, MD | 1 |
| Wadman, RI | 3 |
| Meester-Delver, A | 1 |
| Poll-The, BT | 1 |
| Lemmink, HH | 1 |
| Wokke, JH | 3 |
| van der Pol, WL | 2 |
| van den Berg, LH | 3 |
| Lewelt, A | 1 |
| Scott, C | 1 |
| Maczulski, JA | 1 |
| Stoddard, GJ | 1 |
| Elovic, E | 1 |
| Darbar, IA | 1 |
| Plaggert, PG | 1 |
| Resende, MB | 1 |
| Zanoteli, E | 1 |
| Reed, UC | 1 |
| Dunaway, S | 1 |
| Montes, J | 1 |
| Ryan, PA | 1 |
| Montgomery, M | 1 |
| Sproule, DM | 1 |
| De Vivo, DC | 1 |
| Bosboom, WM | 2 |
| Iannaccone, ST | 2 |
| Vrancken, AF | 2 |
| Sumner, CJ | 1 |
| Huynh, TN | 1 |
| Markowitz, JA | 1 |
| Perhac, JS | 1 |
| Hill, B | 1 |
| Coovert, DD | 1 |
| Schussler, K | 1 |
| Chen, X | 1 |
| Jarecki, J | 1 |
| Burghes, AH | 1 |
| Taylor, JP | 1 |
| Fischbeck, KH | 1 |
| Darras, BT | 1 |
| Kang, PB | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Prospective Evaluation of Infants With Spinal Muscular Atrophy: SPOT SMA[NCT02831296] | 1,000 participants (Anticipated) | Observational [Patient Registry] | 2016-02-29 | Recruiting | |||
| Evaluation of the Muscle Strength and Motor Ability in Children With Spinal Muscle Atrophy Type II and III Treated With Valproic Acid[NCT01033331] | 22 participants (Actual) | Observational | 2006-07-31 | Completed | |||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
3 reviews available for valproic acid and Spinal Muscular Atrophies of Childhood
| Article | Year |
|---|---|
Drug treatment for spinal muscular atrophy types II and III.
Topics: Acetylcarnitine; Adolescent; Amines; Child; Child, Preschool; Creatine; Cyclohexanecarboxylic Acids; | 2011 |
Drug treatment for spinal muscular atrophy types II and III.
Topics: Acetylcarnitine; Adolescent; Amines; Child; Child, Preschool; Creatine; Cyclohexanecarboxylic Acids; | 2012 |
Clinical trials in spinal muscular atrophy.
Topics: Animals; Child; Clinical Trials as Topic; Cyclic AMP Response Element-Binding Protein; Enzyme Inhibi | 2007 |
3 trials available for valproic acid and Spinal Muscular Atrophies of Childhood
| Article | Year |
|---|---|
Clinical trial of L-Carnitine and valproic acid in spinal muscular atrophy type I.
Topics: Action Potentials; Carnitine; Cohort Studies; Drug Therapy, Combination; Female; GABA Agents; Humans | 2018 |
Compound muscle action potential and motor function in children with spinal muscular atrophy.
Topics: Action Potentials; Adolescent; Carnitine; Child; Child, Preschool; Electromyography; Female; GABA Ag | 2010 |
Evaluation of muscle strength and motor abilities in children with type II and III spinal muscle atrophy treated with valproic acid.
Topics: Adolescent; Child; Child, Preschool; Enzyme Inhibitors; Female; Humans; Male; Motor Activity; Muscle | 2011 |
6 other studies available for valproic acid and Spinal Muscular Atrophies of Childhood
| Article | Year |
|---|---|
Modeling the early phenotype at the neuromuscular junction of spinal muscular atrophy using patient-derived iPSCs.
Topics: Animals; Cell Culture Techniques; Cell Differentiation; Cell Line; Coculture Techniques; Humans; Ind | 2015 |
Valproic acid blocks excitability in SMA type I mouse motor neurons.
Topics: Actins; Animals; Anticonvulsants; Axons; Calcium; Calcium Channels; Cell Death; Cells, Cultured; Dos | 2009 |
Genotype-phenotype studies in infantile spinal muscular atrophy (SMA) type I in Germany: implications for clinical trials and genetic counselling.
Topics: Age of Onset; Clinical Trials as Topic; Cohort Studies; Gene Dosage; Genetic Counseling; Genotype; G | 2009 |
Quantification of SMN protein in leucocytes from spinal muscular atrophy patients: effects of treatment with valproic acid.
Topics: Adolescent; Child; Child, Preschool; Enzyme-Linked Immunosorbent Assay; Female; GABA Agents; Humans; | 2011 |
Spinal muscular atrophy type III: trying to understand subtle functional change over time--a case report.
Topics: Anticonvulsants; Child; Early Intervention, Educational; Exhalation; Female; Humans; Longitudinal St | 2012 |
Valproic acid increases SMN levels in spinal muscular atrophy patient cells.
Topics: Anticonvulsants; Cell Line; Child, Preschool; Cyclic AMP Response Element-Binding Protein; Dose-Resp | 2003 |