Page last updated: 2024-10-26

valproic acid and Friedreich Ataxia

valproic acid has been researched along with Friedreich Ataxia in 2 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.

Friedreich Ataxia: An autosomal recessive disease, usually of childhood onset, characterized pathologically by degeneration of the spinocerebellar tracts, posterior columns, and to a lesser extent the corticospinal tracts. Clinical manifestations include GAIT ATAXIA, pes cavus, speech impairment, lateral curvature of spine, rhythmic head tremor, kyphoscoliosis, congestive heart failure (secondary to a cardiomyopathy), and lower extremity weakness. Most forms of this condition are associated with a mutation in a gene on chromosome 9, at band q13, which codes for the mitochondrial protein frataxin. (From Adams et al., Principles of Neurology, 6th ed, p1081; N Engl J Med 1996 Oct 17;335(16):1169-75) The severity of Friedreich ataxia associated with expansion of GAA repeats in the first intron of the frataxin gene correlates with the number of trinucleotide repeats. (From Durr et al, N Engl J Med 1996 Oct 17;335(16):1169-75)

Research Excerpts

ExcerptRelevanceReference
"A 29-year-old female patient with Friedreich's ataxia and partial seizures with acute liver failure during VPA treatment is reported."1.30Fatal liver failure associated with valproate therapy in a patient with Friedreich's disease: review of valproate hepatotoxicity in adults. ( Hehlmann, R; Heubner, C; Holm, E; König, I; König, SA; Schenk, M; Sick, C; Weiss, A, 1999)

Research

Studies (2)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's1 (50.00)18.2507
2000's1 (50.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Herman, D1
Jenssen, K1
Burnett, R1
Soragni, E1
Perlman, SL1
Gottesfeld, JM1
König, SA1
Schenk, M1
Sick, C1
Holm, E1
Heubner, C1
Weiss, A1
König, I1
Hehlmann, R1

Other Studies

2 other studies available for valproic acid and Friedreich Ataxia

ArticleYear
Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia.
    Nature chemical biology, 2006, Volume: 2, Issue:10

    Topics: Acetylation; Alleles; Anilides; Cell Line; Cells, Cultured; Dose-Response Relationship, Drug; Enzyme

2006
Fatal liver failure associated with valproate therapy in a patient with Friedreich's disease: review of valproate hepatotoxicity in adults.
    Epilepsia, 1999, Volume: 40, Issue:7

    Topics: Adolescent; Adult; Chemical and Drug Induced Liver Injury; Comorbidity; Epilepsy; Fatal Outcome; Fem

1999