| Popis: |
Cystic Fibrosis is a genetic disease that results in poor growth manifesting as a reduced fat mass. This study characterized the de novo fatty acid synthesis of both Cftr-null mice and hepatocyte specific Cftr knockouts to determine if this is a potential mechanism resulting in this reduced fat mass. De novo synthesis rates of palmitate and stearate were measured in the liver via isotope labeling of water and mass spectrometry. In addition, the expression of ACC-1 and AMPk were determined via western blotting. Results indicated decreased hepatic fat synthesis in CF males compared to CF females and controls. Enzyme expression results revealed differences in hepatic ACC-1 and AMPk activity levels in both CF genders, which were overall inconclusive. Hepatocyte knockouts showed no difference in palmitate or stearate synthesis and a significant increase in male AMPk expression. Overall, this study supports the notion of an impaired lipid metabolism in CF mice. |
