Increased contents of protein carbonyls were detected by Western blot after derivatization with DNPH in aconitate hydratase (aconitase 2, ACO2), as well as in glial fibrillary acidic protein (GFAP), γ-enolase and ubiquitous mitochondrial creatine kinase (CKMT1A), in a proteomic and enzymatic analysis of human brain post-mortem samples from the striatum and cortex of eight patients with Huntington’s disease (HD) and as many matched controls. This evidence concerns the gene ACO2 and Huntington disease.