CMP-Neu5Ac Hydroxylase Null Mice as a Model for Studying Metabolic Disorders Caused by the Evolutionary Loss of Neu5Gc in Humans

Figure 5

Characterization of mitochondrial dysfunction in liver of Cmah-null mice. (a) Reduction of mitochondrial activity in Cmah-null mice livers, stained using a mitochondrial antibody. (b) The intensity of the fluorescent signal indicates the level of mitochondrial activity. (c) The mtDNA/β-actin ratio, which represents the average copy number, was significantly decreased in Cmah-null mouse-derived livers. The CytB gene amplification level was normalized against the expression of nuclear β-actin. (d) Expression levels of genes involved in mitochondrial functional regulation were determined by RT-qPCR in RNA samples from the livers of WT and Cmah-null mice. Measurements were performed in triplicate, after which the mean expression was calculated and corrected using gapdh expression levels. Error bars indicate standard deviations. Significant differences are indicated by and .

(a)

(b)

(c)

(d)