2023
Tumor suppressor p53 regulates heat shock factor 1 protein degradation in Huntington’s disease
Mansky R, Greguske E, Yu D, Zarate N, Intihar T, Tsai W, Brown T, Thayer M, Kumar K, Gomez-Pastor R. Tumor suppressor p53 regulates heat shock factor 1 protein degradation in Huntington’s disease. Cell Reports 2023, 42: 112198. PMID: 36867535, PMCID: PMC10128052, DOI: 10.1016/j.celrep.2023.112198.Peer-Reviewed Original ResearchConceptsMajor transcription factorTumor suppressor p53Huntington's diseaseHtt aggregationTranscription factorsE3 ligase MDM2Protein degradationSuppressor p53Reciprocal regulationMutant HttP53 stabilizationMolecular differencesCell proliferationHSF1Neurodegenerative diseasesP53Most cancersNeurodegenerationP53 deletionTranscriptionDegradationDeletionAbundanceHTT
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