valproic acid has been researched along with Niemann-Pick Disease, Type C in 4 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.
Niemann-Pick Disease, Type C: An autosomal recessive lipid storage disorder that is characterized by accumulation of CHOLESTEROL and SPHINGOMYELINS in cells of the VISCERA and the CENTRAL NERVOUS SYSTEM. Type C (or C1) and type D are allelic disorders caused by mutation of the NPC1 gene, which encodes a protein that mediates intracellular cholesterol transport from LYSOSOMES. Clinical signs include hepatosplenomegaly and chronic neurological symptoms. Type D is a variant in people with a Nova Scotia ancestry.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Like valproic acid, treatment with primidone reduced free-C levels in LE/LY in NPC1-null/mutant cells." | 5.62 | Beneficial effects of primidone in Niemann-Pick disease type C (NPC)-model cells and mice: Reduction of unesterified cholesterol levels in cells and extension of lifespan in mice. ( Ashikawa, H; Honda, T; Mogi, H; Murayama, T; Nakamura, H, 2021) |
| "Like valproic acid, treatment with primidone reduced free-C levels in LE/LY in NPC1-null/mutant cells." | 1.62 | Beneficial effects of primidone in Niemann-Pick disease type C (NPC)-model cells and mice: Reduction of unesterified cholesterol levels in cells and extension of lifespan in mice. ( Ashikawa, H; Honda, T; Mogi, H; Murayama, T; Nakamura, H, 2021) |
| "Whilst post-ictal psychosis is rarely reported prior to the age of 16, NPC in adolescents and adults is particularly psychotogenic and may increase the risk for post-ictal psychosis in the pediatric population." | 1.36 | Post-ictal psychosis in adolescent Niemann-Pick disease type C. ( Adams, S; Fietz, M; Kornberg, A; Velakoulis, D; Walterfang, M, 2010) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 2 (50.00) | 24.3611 |
| 2020's | 2 (50.00) | 2.80 |
| Authors | Studies |
|---|---|
| Lee, SE | 1 |
| Shin, N | 1 |
| Kook, MG | 1 |
| Kong, D | 1 |
| Kim, NG | 1 |
| Choi, SW | 1 |
| Kang, KS | 1 |
| Ashikawa, H | 1 |
| Mogi, H | 1 |
| Honda, T | 1 |
| Nakamura, H | 1 |
| Murayama, T | 1 |
| Probert, F | 1 |
| Ruiz-Rodado, V | 1 |
| Zhang, X | 1 |
| te Vruchte, D | 1 |
| Claridge, TD | 1 |
| Edgar, M | 1 |
| Tocchio, AZ | 1 |
| Lachmann, RH | 1 |
| Platt, FM | 1 |
| Grootveld, M | 1 |
| Walterfang, M | 1 |
| Kornberg, A | 1 |
| Adams, S | 1 |
| Fietz, M | 1 |
| Velakoulis, D | 1 |
4 other studies available for valproic acid and Niemann-Pick Disease, Type C
| Article | Year |
|---|---|
Human iNSC-derived brain organoid model of lysosomal storage disorder in Niemann-Pick disease type C.
Topics: Autophagy; Biomarkers; Brain; Cell Count; Cell Differentiation; Cell Line; Cell Proliferation; Gene | 2020 |
Beneficial effects of primidone in Niemann-Pick disease type C (NPC)-model cells and mice: Reduction of unesterified cholesterol levels in cells and extension of lifespan in mice.
Topics: Animals; Biological Transport; CHO Cells; Cholesterol; Cricetulus; Disease Models, Animal; Endoplasm | 2021 |
Urinary excretion and metabolism of miglustat and valproate in patients with Niemann-Pick type C1 disease: One- and two-dimensional solution-state (1)H NMR studies.
Topics: 1-Deoxynojirimycin; Animals; Cohort Studies; Enzyme Inhibitors; Humans; Magnetic Resonance Spectrosc | 2016 |
Post-ictal psychosis in adolescent Niemann-Pick disease type C.
Topics: Adolescent; Age Factors; Anticonvulsants; Cells, Cultured; Cholesterol Esters; Esterification; Fibro | 2010 |