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oai:recercat.cat:10459.1/571612024-12-05T21:21:13Zcom_2072_3622col_2072_479130
RECERCAT author Sorolla Bardají, Maria Alba author Reverter Branchat, Gemma author Tamarit Sumalla, Jordi author Ferrer, Isidre author Ros Salvador, Joaquim author Cabiscol Català, Elisa 2024-12-05T21:21:13Z 2024-12-05T21:21:13Z 2016-06-06T10:32:52Z2025-01-012008 http://hdl.handle.net/10459.1/57161 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) (c) Elsevier Inc., 2008 info:eu-repo/semantics/restrictedAccess Huntington disease Proteomic analysis Oxidative stress Protein carbonylation Proteomic and oxidative stress analysis in human brain samples of Huntington disease article publishedVersion