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2024-12-05T21:21:13Z urn:hdl:10459.1/57161 Proteomic and oxidative stress analysis in human brain samples of Huntington disease Sorolla Bardají, Maria Alba Reverter Branchat, Gemma Tamarit Sumalla, Jordi Ferrer, Isidre Ros Salvador, Joaquim Cabiscol Català, Elisa Huntington disease Proteomic analysis Oxidative stress Protein carbonylation Huntington disease (HD) is a neurodegenerative disorder caused by expansion of CAG repeats in exon 1 of the huntingtin gene, affecting initially the striatum and progressively the cortex. This work reports a proteomic analysis of human brain postmortem samples obtained from striatum and cortex of patients with HD compared to samples of age- and sex-matched controls. Antioxidant defense proteins that were strongly induced in striatum, but also detectable in cortex, were identified as peroxiredoxins 1, 2, and 6, as well as glutathione peroxidases 1 and 6. The activities of other antioxidant enzymes such as mitochondrial superoxide dismutase and catalase were also increased in HD. Aconitase, a protein involved in energy metabolism, showed decreased activities in striatum of HD patients. Protein carbonyls, used as markers of oxidative stress, were increased in HD, and glial fibrillary acidic protein, aconitase, γ-enolase, and creatine kinase B were identified as the main targets. Taken together, these results indicate that oxidative stress and damage to specific macromolecules would participate in the disease progression. Also, these data support the rationale for therapeutic strategies that either potentiate antioxidant defenses or avoid oxidative stress generation to delay disease progression. This work has been supported by Grants BFU2004-00593/BMC, BFU2007-66249, and CSD2007-20 Consolider Ingenio 2010 from the Ministerio de Educación y Ciencia (Spain) and SGR2005-00677 from the Generalitat de Catalunya. M. A. Sorolla is the recipient of a Ph.D. fellowship from the Ministerio de Educación y Ciencia (Spain) 2024-12-05T21:21:13Z 2024-12-05T21:21:13Z 2016-06-06T10:32:52Z 2025-01-01 2008 article publishedVersion http://hdl.handle.net/10459.1/57161 MIECI/PN2004-2007/BFU2004-00593/BMC MIECI/PN2004-2007/BFU2007-66249 Reproducció del document publicat a https://doi.org/10.1016/j.freeradbiomed.2008.05.014 Free Radical Biology and Medicine, 2008, vol. 45, núm. 5, p. 667-678 (c) Elsevier Inc., 2008 info:eu-repo/semantics/restrictedAccess Elsevier