Oxidative Stress in Spinocerebellar Ataxia Type 7 Is Associated with Disease Severity

• Published in: Cerebellum (London, England) Vol. 17; no. 5; pp. 601 - 609
• Main Authors: Torres-Ramos, Y., Montoya-Estrada, A., Cisneros, B., Tercero-Pérez, K., León-Reyes, G., Leyva-García, N., Hernández-Hernández, Oscar, Magaña, Jonathan J.
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.10.2018; Springer Nature B.V
• Subjects: Adult; Antioxidants; Ataxia; Ataxin; Autophagy; Biomarkers - blood; Biomedical and Life Sciences; Biomedicine; Carbonyl compounds; Case-Control Studies; Cross-Sectional Studies; Female; Gene regulation; Glutathione peroxidase; Glutathione reductase; Humans; Lipid peroxidation; Lipids; Male; Malondialdehyde; Middle Aged; Neural coding; Neurobiology; Neurodegeneration; Neurodegenerative diseases; Neurology; Neurosciences; Original Paper; Oxidation; Oxidative Stress; Paraoxonase; Pathogenesis; Phagocytosis; Polyglutamine; Polyglutamine diseases; Protein structure; Proteins; Severity of Illness Index; Spinocerebellar ataxia; Spinocerebellar Ataxias - blood; Transcription; Trinucleotide repeat diseases; Oxidative stress; Protein oxidation; Spinocerebellar ataxia type 7; Lipid oxidation; Blood biomarkers; Antioxidant defense

Spinocerebellar ataxia type 7 is a neurodegenerative inherited disease caused by a CAG expansion in the coding region of the ATXN7 gene, which results in the synthesis of polyglutamine-containing ataxin-7. Expression of mutant ataxin-7 disturbs different cell processes, including transcriptional reg...