2013
UCP2 overexpression worsens mitochondrial dysfunction and accelerates disease progression in a mouse model of amyotrophic lateral sclerosis
Peixoto PM, Kim HJ, Sider B, Starkov A, Horvath TL, Manfredi G. UCP2 overexpression worsens mitochondrial dysfunction and accelerates disease progression in a mouse model of amyotrophic lateral sclerosis. Molecular And Cellular Neuroscience 2013, 57: 104-110. PMID: 24141050, PMCID: PMC3891658, DOI: 10.1016/j.mcn.2013.10.002.Peer-Reviewed Original ResearchConceptsAmyotrophic lateral sclerosisDouble transgenic miceFamilial amyotrophic lateral sclerosisMouse modelLateral sclerosisMitochondrial dysfunctionTransgenic miceMutant SOD1 mouse modelHuman UCP2Brain mitochondriaSOD1 mutant miceUCP2 overexpressionPotential neuroprotective effectsProtection of neuronsSOD1 mouse modelCentral nervous systemReactive oxygen species productionDisease courseG93A miceNeuroprotective effectsNeuroprotective roleFree radical generationDisease progressionOxygen species productionInjury paradigms
2011
GPA protects the nigrostriatal dopamine system by enhancing mitochondrial function
Horvath TL, Erion DM, Elsworth JD, Roth RH, Shulman GI, Andrews ZB. GPA protects the nigrostriatal dopamine system by enhancing mitochondrial function. Neurobiology Of Disease 2011, 43: 152-162. PMID: 21406233, PMCID: PMC3623269, DOI: 10.1016/j.nbd.2011.03.005.Peer-Reviewed Original ResearchConceptsNormal chow-fed miceNigrostriatal dopamine systemChow-fed miceTH neuronsGuanidinopropionic acidNormal chowParkinson's diseaseDopamine systemMitochondrial functionMitochondrial dysfunctionModels of neurodegenerationMitochondrial numberAMPK activityMPTP treatmentMPTP intoxicationNigrostriatal functionNeuroprotective effectsMitochondrial respirationNeuroprotective propertiesStriatal dopamineAMPK-dependent increaseDisease progressionMouse modelMiceMPTP