In contrast to transgenic models of Huntington’s disease (HD) newly available knock-in models, such as the HttQ175, recapitulate the heterozygosity present in the clinically observed pathophysiology. Here we report a longitudinal assessment of striatal metabolites using 1H-MRS and volumetry in 30+ brain regions in both male and female HttQ175 mice. Apart from a genotype-specific, progressive neurodegenerative phenotype present in both genders, we discovered an entirely novel feature in mouse models of HD, namely individuals with abnormally high glutamine (Gln) levels, whose incidence increased with mutant Htt gene dosage. In line with emerging data on a widespread peripheral metabolic perturbation in HD, we hypothesize that this excessive Gln is a consequence of disordered hepatic metabolism.
How to access this content:
For one year after publication, abstracts and videos are only open to registrants of this annual meeting. Registrants should use their existing login information. Non-registrant access can be purchased via the ISMRM E-Library.
After one year, current ISMRM & ISMRT members get free access to both the abstracts and videos. Non-members and non-registrants must purchase access via the ISMRM E-Library.
After two years, the meeting proceedings (abstracts) are opened to the public and require no login information. Videos remain behind password for access by members, registrants and E-Library customers.
Keywords