Aberrant A sub(2A) receptor function in peripheral blood cells in Huntington's disease

Huntington's disease (HD) is a progressively disabling neurodegenerative disease caused by an expanded CAG mutation in the gene encoding huntingtin (Htt). There is increasing interest in the identification of peripheral biomarkers of the disease that could be used to predict its progression and...

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Published in:The FASEB journal 2003-11, Vol.17 (14), p.2148-2150
Main Authors: Varani, Katia, Abbracchio, Maria P, Cannella, Milena, Cislaghi, Giuliana, Giallonardo, Patrizia, Mariotti, Caterina, Cattabriga, Elena, Cattabeni, Flaminio, Borea, Pier Andrea, Squitieri, Ferdinando, Cattaneo, Elena
Format: Article
Language:English
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Summary:Huntington's disease (HD) is a progressively disabling neurodegenerative disease caused by an expanded CAG mutation in the gene encoding huntingtin (Htt). There is increasing interest in the identification of peripheral biomarkers of the disease that could be used to predict its progression and/or to monitor the efficacy of upcoming therapeutic treatments. A sub(2A) adenosine receptors are specifically found on striatal medium spiny neurons undergoing death in HD, suggesting they may play a role in HD-associated neurodegeneration. In a previous study we demonstrated the presence of an aberrantly increased A sub(2A) adenosine receptor function in immortalized striatal cells engineered to express mutant Htt. The present study represents the follow-up of that original observation and was aimed at testing whether a similar dysfunction is present in the peripheral blood cells (platelets, lymphocytes, and neutrophils) of human subjects carrying the mutant HD gene.
ISSN:0892-6638
DOI:10.1096/fj.03-0079fje