Proteomic and oxidative stress analysis in human brain samples of Huntington disease.

摘要: Huntington disease (HD) is a neurodegenerative disorder caused by expansion of CAG repeats in exon 1 the huntingtin gene, affecting initially striatum and progressively cortex. This work reports proteomic analysis human brain postmortem samples obtained from cortex patients with HD compared to age- sex-matched controls. Antioxidant defense proteins that were strongly induced striatum, but also detectable cortex, identified as peroxiredoxins 1, 2, 6, well glutathione peroxidases 6. The activities other antioxidant enzymes such mitochondrial superoxide dismutase catalase increased HD. Aconitase, protein involved energy metabolism, showed decreased patients. Protein carbonyls, used markers oxidative stress, HD, glial fibrillary acidic protein, aconitase, γ-enolase, creatine kinase B main targets. Taken together, these results indicate stress damage specific macromolecules would participate progression. Also, data support rationale for therapeutic strategies either potentiate defenses or avoid generation delay