2023
Animal models of Huntington’s disease and their applicability to novel drug discovery and development
Upadhayay S, Jamwal S, Kumar P. Animal models of Huntington’s disease and their applicability to novel drug discovery and development. Expert Opinion On Drug Discovery 2023, 18: 527-538. PMID: 37042034, DOI: 10.1080/17460441.2023.2201493.Peer-Reviewed Original ResearchConceptsHD animal modelsAnimal modelsHuntington's diseaseNon-motor symptomsProgressive neurodegenerative disorderDrug therapyDisease progressionPreclinical phaseLoss of functionNeurodegenerative disordersClinical phaseDrug discoveryDiseaseNovel drug discoveryHuntingtin geneToxic gainCAG trinucleotide repeatDrugsProgressionHD geneEnormous progression
2019
Mitochondrial Dysfunction in Huntington’s Disease; Interplay Between HSF1, p53 and PGC-1α Transcription Factors
Intihar TA, Martinez EA, Gomez-Pastor R. Mitochondrial Dysfunction in Huntington’s Disease; Interplay Between HSF1, p53 and PGC-1α Transcription Factors. Frontiers In Cellular Neuroscience 2019, 13: 103. PMID: 30941017, PMCID: PMC6433789, DOI: 10.3389/fncel.2019.00103.Peer-Reviewed Original ResearchMutant HttTranscription factorsMitochondrial dysfunctionPGC-1αMitochondrial functionCo-activator PGC-1αTranscription factor HSF1Main transcription factorHuntington's diseaseProtein homeostasisHSF1Medium spiny neuronsP53 levelsUnifying mechanismHuntingtin geneNeurodegenerative diseasesP53ProteinPotential roleStriatal medium spiny neuronsCAG repeatsThird mechanismMisfoldHD progressionGenes
2010
Identification and Evaluation of Small Molecule Pan-Caspase Inhibitors in Huntington's Disease Models
Leyva MJ, DeGiacomo F, Kaltenbach LS, Holcomb J, Zhang N, Gafni J, Park H, Lo DC, Salvesen GS, Ellerby LM, Ellman JA. Identification and Evaluation of Small Molecule Pan-Caspase Inhibitors in Huntington's Disease Models. Cell Chemical Biology 2010, 17: 1189-1200. PMID: 21095569, PMCID: PMC3035168, DOI: 10.1016/j.chembiol.2010.08.014.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsApoptosisCaspase 3Caspase 6Caspase InhibitorsCells, CulturedCoumarinsCysteine Proteinase InhibitorsDisease Models, AnimalHumansHuntingtin ProteinHuntington DiseaseMiceNerve Tissue ProteinsNeuronsNuclear ProteinsRatsSmall Molecule LibrariesStructure-Activity RelationshipSubstrate SpecificityConceptsHuntington's diseaseHtt proteolysisPan-caspase inhibitorHD therapeutic developmentDisease modelsPostmortem human tissueMouse HD modelsCaspase-3HD modelProteolysis of httHuntington's disease modelsCritical pathological eventCortical neuronsTherapeutic targetPathological eventsPromising targetTherapeutic developmentHuntingtin geneInhibitorsCell deathDiseaseIrreversible inhibitorHuman tissuesHTT proteinScreening method
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