Gregorczyk Magdalena, Mika Adriana, Śledziński Tomasz, Tomczyk Marta, Rybakowska Iwona
Department of Biochemistry and Clinical Physiology, Medical University of Gdansk, 80-210 Gdansk, Poland.
Department of Environmental Analysis, Faculty of Chemistry, University of Gdansk, 80-309 Gdansk, Poland.
Int J Mol Sci. 2025 Jul 28;26(15):7304. doi: 10.3390/ijms26157304.
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-old mice 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). In mice, 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 in mice. Overall, our results indicate that mice exhibit more pronounced alterations in hepatic FA profiles than mice, suggesting that early-onset HD may be associated with more severe peripheral metabolic dysregulation.
亨廷顿舞蹈症（HD）的特征是进行性神经退行性变，但越来越多的证据表明其存在多系统受累，包括小儿HD早期的肝脂肪变性。因此，考虑全身改变，尤其是肝脏脂质代谢方面的改变很重要。在本研究中，我们分析了两种有症状的HD小鼠模型的脂肪酸（FA）谱：代表早发性HD的2月龄小鼠和代表晚发性HD的22月龄（）小鼠，以及年龄匹配的野生型（）对照。通过气相色谱 - 质谱联用（GC - MS）评估肝脏组织中的FA组成。与野生型相比，在小鼠中，我们观察到总异支链、单不饱和和n - 6多不饱和脂肪酸水平升高。相比之下，只有少数FA种类在小鼠中浓度降低。总体而言，我们的结果表明，与小鼠相比，小鼠肝脏FA谱的改变更为明显，这表明早发性HD可能与更严重的外周代谢失调有关。
