Page last updated: 2024-11-03

primidone and Niemann-Pick Disease, Type C

primidone has been researched along with Niemann-Pick Disease, Type C in 1 studies

Primidone: A barbiturate derivative that acts as a GABA modulator and anti-epileptic agent. It is partly metabolized to PHENOBARBITAL in the body and owes some of its actions to this metabolite.
primidone : A pyrimidone that is dihydropyrimidine-4,6(1H,5H)-dione substituted by an ethyl and a phenyl group at position 5. It is used as an anticonvulsant for treatment of various types of seizures.

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.

Research Excerpts

ExcerptRelevanceReference
"Like valproic acid, treatment with primidone reduced free-C levels in LE/LY in NPC1-null/mutant cells."5.62Beneficial 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."1.62Beneficial 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)

Research

Studies (1)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's1 (100.00)2.80

Authors

AuthorsStudies
Ashikawa, H1
Mogi, H1
Honda, T1
Nakamura, H1
Murayama, T1

Other Studies

1 other study available for primidone and Niemann-Pick Disease, Type C

ArticleYear
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.
    European journal of pharmacology, 2021, Apr-05, Volume: 896

    Topics: Animals; Biological Transport; CHO Cells; Cholesterol; Cricetulus; Disease Models, Animal; Endoplasm

2021