Quantitative metabolic profiling of alpha-keto acids in Friedreich's ataxia

The plasma distribution of alpha-keto acids was measured in 26 subjects including 8 patients with Friedreich's ataxia, 8 with the recessive spastic ataxia of Charlevoix-Sageunay and 10 healthy volunteers. The groups were matched with regards to age, sex, weight and the study was conducted under standardized dietary intake. The result indicate significant differences in the alpha-keto acids distribution between the groups.