Fatty Acid Profile in the Liver of Mice with Early- and Late-Onset Forms of Huntington's Disease.

Abstract

Huntington's disease (HD) is characterized by progressive neurodegeneration, but increasing evidence points to multisystemic involvement, including early hepatic steatosis in pediatric HD. Therefore, it is important to consider systemic alterations, particularly in liver lipid metabolism. In this study, we analyzed fatty acid (FA) profiles in two symptomatic HD mouse models: 2-month-oldmice representing early-onset HD and 22-month-old() mice representing late-onset HD, along with age-matched wild-type () controls. FA composition in liver tissue was assessed by gas chromatography-mass spectrometry (GC-MS). Inmice, we observed increased levels of total iso-branched chain, monounsaturated, and n-6 polyunsaturated FAs compared to WT. In contrast, only a few FA species showed reduced concentrations inmice. Overall, our results indicate thatmice exhibit more pronounced alterations in hepatic FA profiles thanmice, suggesting that early-onset HD may be associated with more severe peripheral metabolic dysregulation.