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Duplication 15q syndrome (Dup15q) is an autism-associated disorder co-incident with high rates of pediatric epilepsy. Additional copies of the E3 ubiquitin ligase UBE3A are thought to cause Dup15q phenotypes, yet models overexpressing UBE3A in neurons have not recapitulated the epilepsy phenotype. We show that Drosophila endogenously expresses Dube3a (fly UBE3A homolog) in glial cells and neurons, prompting an investigation into the consequences of glial Dube3a overexpression. Here we expand on previous work showing that the Na+/K+ pump ATPα is a direct ubiquitin ligase substrate of Dube3a. A robust seizure-like phenotype was observed in flies overexpressing Dube3a in glial cells, but not neurons. Glial-specific knockdown of ATPα also produced seizure-like behavior, and this phenotype was rescued by simultaneously overexpressing ATPα and Dube3a in glia. Our data provides the basis of a paradigm shift in Dup15q research given that clinical phenotypes have long been assumed to be due to neuronal UBE3A overexpression.
Pubmed ID: 28888970 RIS Download
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View all literature mentionsDrosophila melanogaster with name w[1118]; P{w[+m*]=GAL4}repo/TM3, Sb[1] from BDSC.
View all literature mentionsThis monoclonal targets Bruchpilot
View all literature mentionsDrosophila melanogaster with name M{UAS-Atpalpha.ORF}ZH-86Fb from Flybase.
View all literature mentionsDrosophila melanogaster with name w[*]; P{y[+t7.7] w[+mC]=10XUAS-IVS-myr::tdTomato}attP40 from BDSC.
View all literature mentionsDrosophila melanogaster with name y[1] sc[*] v[1] sev[21]; P{y[+t7.7] v[+t1.8]=TRiP.HMS00703}attP2 from BDSC.
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View all literature mentionsThis monoclonal targets POLG
View all literature mentionsThis polyclonal targets GAPDH - Loading Control
View all literature mentionsThis monoclonal targets elav Drosophila protein; embryonic lethal abnormal vision
View all literature mentionsDrosophila melanogaster with name w[*]; P{y[+t7.7] w[+mC]=10XUAS-IVS-myr::tdTomato}attP40 from BDSC.
View all literature mentionsDrosophila melanogaster with name M{UAS-Atpalpha.ORF}ZH-86Fb from Flybase.
View all literature mentionsDrosophila melanogaster with name y[1] sc[*] v[1] sev[21]; P{y[+t7.7] v[+t1.8]=TRiP.HMS00703}attP2 from BDSC.
View all literature mentionsThis polyclonal targets IgG
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View all literature mentionsThis monoclonal targets Repo; Reversed polarity protein
View all literature mentionsThis unknown targets IgG
View all literature mentionsThis unknown targets Mouse IgG (H+L)
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis polyclonal targets IgG (H+L)
View all literature mentionsThis monoclonal targets Mouse Drosophila fasciclin II
View all literature mentionsThis polyclonal targets GFP
View all literature mentionsThis unknown targets IgG
View all literature mentionsDrosophila melanogaster with name w[1118]; P{w[+m*]=GAL4}repo/TM3, Sb[1] from BDSC.
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