A Mouse Model of L-2-Hydroxyglutaric Aciduria, a Disorder of Metabolite Repair
Fig 3
L-2-hydroxyglutarate formation in l2hgdh-/- cells is decreased if mitochondrial malate dehydrogenase (mMDH) is knocked down.
MEF cells derived from l2hgdh+/+ and l2hgdh-/- 14.5 day-embryos were treated with control (scrambled) or mMDH-specific siRNAs for 48 h. (A) mMDH immunoreactivity was determined in lysates of cells treated or not with siRNA. (B) mMDH activity in cell lysates was determined by separating this enzyme from cytoplasmic MDH by cation exchange chromatography. (C) (D+L)-2-hydroxyglutarate concentration was determined in the medium by GC-MS.