Effect of Trehalose on the Properties of Mutant γPKC, Which Causes Spinocerebellar Ataxia Type 14, in Neuronal Cell Lines and Cultured Purkinje Cells*
Seki T, Abe-Seki N, Kikawada T, Takahashi H, Yamamoto K, Adachi N, Tanaka S, Hide I, Saito N, Sakai N. Effect of Trehalose on the Properties of Mutant γPKC, Which Causes Spinocerebellar Ataxia Type 14, in Neuronal Cell Lines and Cultured Purkinje Cells*. Journal Of Biological Chemistry 2010, 285: 33252-33264. PMID: 20705605, PMCID: PMC2963337, DOI: 10.1074/jbc.m110.146704.Peer-Reviewed Original ResearchConceptsPrimary cultured Purkinje cellsApoptotic cell deathSH-SY5Y cellsNeuronal cell lineCell deathDevelopment of dendritesSH-SY5YSpinocerebellar ataxia type 14Autosomal dominant neurodegenerative diseaseCell linesDominant neurodegenerative diseaseModel of Huntington's diseaseSusceptible to aggregationAnimal models of Huntington's diseaseMissense mutationsPrion diseasesHuntington's diseaseProtein turnoverType 14Spinocerebellar ataxia typeDisaccharide trehaloseAlzheimer's diseaseNeurodegenerative diseasesCellular dysfunctionTrehaloseCongo Red, an Amyloid-Inhibiting Compound, Alleviates Various Types of Cellular Dysfunction Triggered by Mutant Protein Kinase Cγ That Causes Spinocerebellar Ataxia Type 14 (SCA14) by Inhibiting Oligomerization and Aggregation
Seki T, Takahashi H, Yamamoto K, Ogawa K, Onji T, Adachi N, Tanaka S, Hide I, Saito N, Sakai N. Congo Red, an Amyloid-Inhibiting Compound, Alleviates Various Types of Cellular Dysfunction Triggered by Mutant Protein Kinase Cγ That Causes Spinocerebellar Ataxia Type 14 (SCA14) by Inhibiting Oligomerization and Aggregation. Journal Of Pharmacological Sciences 2010, 114: 206. PMID: 20938103, DOI: 10.1254/jphs.10170fp.Peer-Reviewed Original ResearchMeSH KeywordsAmyloidAnimalsApoptosisCells, CulturedCerebellumColoring AgentsCongo RedDendritesDose-Response Relationship, DrugEmbryo, MammalianHumansMiceMice, Inbred ICRMutant ProteinsMutation, MissenseNeuroblastomaNeurodegenerative DiseasesProtein Kinase CPurkinje CellsSpinocerebellar AtaxiasSpinocerebellar DegenerationsConceptsGreen fluorescent proteinApoptotic cell deathCell deathFibril formationFormation of misfolded proteinsSpinocerebellar ataxia type 14Autosomal dominant neurodegenerative diseaseDominant neurodegenerative diseaseAggregate formationSH-SY5Y cellsAmyloid oligomersCongo redSusceptible to aggregationMissense mutationsFluorescent proteinType 14SH-SY5YDevelopment of dendritesSCA14Spinocerebellar ataxia typeNeurodegenerative diseasesHistological dyesCellular dysfunctionExpression levelsProtein
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