Figure 3.

Organic aciduria and HMG-CoA lyase deficiency in Sod2−/− mice. (a) Representative gas chromatogram of a 9-day-old Sod2−/− mouse urine (blue) superimposed on a chromatogram from a 9-day-old control (red). Two of the abnormal metabolites associated with HMG-CoA lyase deficiency are indicated (peaks 2 and 3), and the increased levels of succinic and citric acids are shown (peaks 1 and 4). IS, internal standards; peak 1, succinic acid; peak 2, 3-OH-3-methyl glutaric acid; peak 3, 3-methyl glutaconic acid; peak 4, citric acid. Peak identities were confirmed by MS. (b) HMG-CoA lyase activity in isolated mitochondria from livers of 9- to 10-day-old Sod2−/− mice. n = 5 for Sod2−/− animals (closed bars); n = 6 for wild-type animals (open bars).