Article
Expression of Idh1R132H in the Murine Subventricular Zone Stem Cell Niche Recapitulates Features of Early Gliomagenesis.
Cancer cell - 10 Oct 2016
Bardella Chiara, Al-Dalahmah Osama, Krell Daniel, Brazauskas Pijus, Al-Qahtani Khalid, Tomkova Marketa, Adam Julie, Serres Sébastien, Lockstone Helen, Freeman-Mills Luke, Pfeffer Inga, Sibson Nicola, Goldin Robert, Schuster-Böeckler Benjamin, Pollard Patrick J, Soga Tomoyoshi, McCullagh James S, Schofield Christopher J, Mulholland Paul, Ansorge Olaf, Kriaucionis Skirmantas, Ratcliffe Peter J, Szele Francis G, Tomlinson Ian
Abstract excerpt
Isocitrate dehydrogenase 1 mutations drive human gliomagenesis, probably through neomorphic enzyme activity that produces D-2-hydroxyglutarate. To model this disease, we conditionally expressed Idh1R132H in the subventricular zone (SVZ) of the adult mouse brain. The mice developed hydrocephalus and grossly dilated lateral ventricles, with accumulation of 2-hydroxyglutarate and reduced α-ketoglutarate. Stem and...
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