Changes of A1 adenosine receptors (A1AR) in Huntington’s disease

Huntington’s disease (HD) is a neurodegenerative disorder caused by a CAG trinucleotide repeat expansion in the HD gene of autosomal dominant inheritance penetrating typically around the age of 40. Typical symptoms are choreatic movements and behavioural symptoms. Life expectancy is reduced in HD patients. In cooperation with the Huntington Centre at St. Josef University Hospital Bochum we studied changes of the A1AR at 8 manifest and 15 pre-manifest HD gene carriers versus 36 controls. Using PET and the radioligand [18F]CPFPX (developed in house by the Institute of Radiochemistry) we observed a global upregulation of the A1AR in early pemanifest gene carriers while A1AR availability decreased considerably in manifest patients (especially in the striatum). The amygdale was identified as the structure which showed the earliest changes.

Figure: Statistical parametric mapping of [18F]CPFPX binding potential (BPND) in 8 patients with manifest Huntington’s disease vs. 21 healthy age normalized controls. Pixel wise T-values are colour-coded (blue-green symbolizes a decrease and red-yellow an increase compared to controls).

The study has been published in:

Matusch A, Saft C, Elmenhorst D, Kraus PH, Gold R, Hartung HP, Bauer A (2014). Cross sectional PET study of cerebral adenosine A1 receptors in premanifest and manifest Huntington's disease. Eur J Nucl Med Mol Imaging. 41(6):1210-1220.

http://link.springer.com/article/10.1007%2Fs00259-014-2724-8