By characterizing the Alb-Cre-Fxn L3/L− mice at 4 weeks of age, we observed a strongly affected liver, displaying the classic hallmarks of frataxin-deficient tissue such as abnormal mitochondrial structures, deficit of Fe-S cluster-dependent activities and intramitochondrial electron-dense deposits reminiscent of iron deposits.
Clusters of normal hepatocytes integrated in the host liver, displaying a normal histology; however, transplanted nodular hepatocytes formed new hepatocyte nodules, with altered morphology and sharp demarcation from surrounding host liver.
In our study, we found the sHPSs showed tissue specific responses with sHSPs in the liver displaying the greatest magnitude change.
The kidney cortex, kidney medulla, and liver displayed a large spread among affected cellular functions through the day.
Isolated mitochondria from MSD liver displayed a striking decrease in ATP content (Fig. 2D).
WWOX expression in normal liver tissue was also confirmed by immunohistochemistry. Hepatocytes from five normal liver displayed a strong cytoplasmic staining for WWOX protein as shown in Figure 5A.
We focused on hepatic mRNA levels because liver displays a broad spectrum of mRNAs that are responsive to dioxins and/or to AHR genotype [ 20] and because liver is a prime site of dioxin toxicity, displaying many phenotypic differences between sensitive and resistant rats [ 29].
Therefore, using the entire set of 150 probes, PAM predicted the inflammation group in 8 of the 9 livers with predominant inflammation scores by histology; the remaining liver displayed a signature typical of the fibrosis group (infant 4 in Figure 2a, b).
Interestingly, also the native (nCK2) tetrameric enzyme purified from rat liver displays a negligible residual activity (6%) when treated with 1 μM quinalizarin consistent with the concept that in these native preparations by far predominant form of CK2 is the holoenzyme, while the isolated catalytic subunits must be nearly absent.
Mice with a severe HNF4α deficiency in the liver displayed reduced growth, altered glucose homeostasis and a postnatal lethal phenotype.
