2022
LINE-1 activation in the cerebellum drives ataxia
Takahashi T, Stoiljkovic M, Song E, Gao XB, Yasumoto Y, Kudo E, Carvalho F, Kong Y, Park A, Shanabrough M, Szigeti-Buck K, Liu ZW, Kristant A, Zhang Y, Sulkowski P, Glazer PM, Kaczmarek LK, Horvath TL, Iwasaki A. LINE-1 activation in the cerebellum drives ataxia. Neuron 2022, 110: 3278-3287.e8. PMID: 36070749, PMCID: PMC9588660, DOI: 10.1016/j.neuron.2022.08.011.Peer-Reviewed Original ResearchConceptsLINE-1 activationL1 activationAtaxia telangiectasia patientsNuclear element-1Transposable elementsEpigenetic silencersHuman genomeL1 promoterMolecular regulatorsDNA damagePurkinje cell dysfunctionElement 1First direct evidenceTelangiectasia patientsDirect targetingCerebellar expressionNeurodegenerative diseasesDisease etiologyCalcium homeostasis
2009
Absence of autophagy results in reactive oxygen species-dependent amplification of RLR signaling
Tal MC, Sasai M, Lee HK, Yordy B, Shadel GS, Iwasaki A. Absence of autophagy results in reactive oxygen species-dependent amplification of RLR signaling. Proceedings Of The National Academy Of Sciences Of The United States Of America 2009, 106: 2770-2775. PMID: 19196953, PMCID: PMC2650341, DOI: 10.1073/pnas.0807694106.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAutophagyAutophagy-Related Protein 5Cells, CulturedDEAD Box Protein 58DEAD-box RNA HelicasesDNA, MitochondrialEnzyme-Linked Immunosorbent AssayFlow CytometryInterferon Type IMacrophagesMiceMicrotubule-Associated ProteinsMitochondriaReactive Oxygen SpeciesReverse Transcriptase Polymerase Chain ReactionSignal TransductionConceptsReactive oxygen speciesDysfunctional mitochondriaInnate antiviral defenseAntiviral defenseKey antiviral cytokinesAbsence of autophagyMitochondrial reactive oxygen speciesHomeostatic regulationRole of autophagyTreatment of cellsIPS-1RLR signalingVesicular stomatitis virusAutophagy resultsRNA virusesWT cellsMitochondriaAutophagyType I IFNStomatitis virusRLRLike receptorsOxygen speciesNeurodegenerative diseasesInflammatory disorders