The Daam2–VHL–Nedd4 axis governs developmental and regenerative oligodendrocyte differentiation
Ding X, Jo J, Wang C, Cristobal C, Zuo Z, Ye Q, Wirianto M, Lindeke-Myers A, Choi J, Mohila C, Kawabe H, Jung S, Bellen H, Yoo S, Lee H. The Daam2–VHL–Nedd4 axis governs developmental and regenerative oligodendrocyte differentiation. Genes & Development 2020, 34: 1177-1189. PMID: 32792353, PMCID: PMC7462057, DOI: 10.1101/gad.338046.120.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCell DifferentiationGene Expression Regulation, DevelopmentalHumansMiceMice, KnockoutMicrofilament ProteinsMultiple SclerosisMyelin SheathNedd4 Ubiquitin Protein LigasesNerve RegenerationNervous System DiseasesOligodendrogliaProtein StabilityRho GTP-Binding ProteinsUbiquitinationVon Hippel-Lindau Tumor Suppressor ProteinConceptsWhite matter injuryUbiquitin-proteasomal systemGenetic knockout mouse modelsOligodendrocyte differentiationWhite matter lesionsKnockout mouse modelDemyelination modelMultiple sclerosisDisease-driving proteinsMatter lesionsOligodendrocyte dysfunctionPathogenic accumulationMouse modelDevelopmental myelinationNeurological disordersGlial biologyOligodendrocyte developmentE3 ligase VHLVHLRepairE3 ubiquitinProteomic analysisRemyelinationSclerosisPatientsLoss- or Gain-of-Function Mutations in ACOX1 Cause Axonal Loss via Different Mechanisms
Chung H, Wangler M, Marcogliese P, Jo J, Ravenscroft T, Zuo Z, Duraine L, Sadeghzadeh S, Li-Kroeger D, Schmidt R, Pestronk A, Rosenfeld J, Burrage L, Herndon M, Chen S, Network M, Shillington A, Vawter-Lee M, Hopkin R, Rodriguez-Smith J, Henrickson M, Lee B, Moser A, Jones R, Watkins P, Yoo T, Mar S, Choi M, Bucelli R, Yamamoto S, Lee H, Prada C, Chae J, Vogel T, Bellen H. Loss- or Gain-of-Function Mutations in ACOX1 Cause Axonal Loss via Different Mechanisms. Neuron 2020, 106: 589-606.e6. PMID: 32169171, PMCID: PMC7289150, DOI: 10.1016/j.neuron.2020.02.021.Peer-Reviewed Original ResearchConceptsSchwann cellsAxonal lossMurine Schwann cellsPrimary Schwann cellsTreatment of fliesLong-chain fatty acid β-oxidation pathwayNeuronal lossGlial lossSynaptic transmissionRate-limiting enzymeDevelopmental delayACOX1Elevated levelsFatty acid β-oxidation pathwayReactive oxygen speciesDifferent mechanismsPupal deathPatientsDominant variantFunction mutationsGliaOxygen speciesTreatmentDe novoCells