Figure 6. Probucol supplementation increases content of CoQ₉ and CoQ₁₀ in kidney and liver of Pdss2^kd/kd missense mutant mice.

Long-term probucol supplementation

A–D. Pdss2^kd/kd mice (3 females and 5 males) were fed diets supplemented with probucol (1% w/w) from birth and killed at 205–207 days of age. Control B6.Pdss2^kd/kd mice (2 females and 2 males) were fed unsupplemented standard chow diet and sacrificed at 205–215 days of age. Each bar represents the average content of (A) CoQ₉ and CoQ₉H₂ and (B) CoQ₁₀ and CoQ₁₀H₂ measured in three aliquots of liver homogenates prepared from each mouse, calculated as the average of six measurements from three independent sample aliquots. Error bars indicate SD. Twenty-four hour urine albumin levels collected immediately prior to liver dissection are indicated for each animal. (C) Total CoQ₉ content represents the average ± SD of the sum of CoQ₉ plus CoQ₉H₂ (determined from panel A). (D) Total CoQ₁₀ content represents the average ± SD of the sum of CoQ₁₀ and CoQ₁₀H₂ (determined from panel B) *p < 0.05 and ***p < 0.001.

Short-term probucol supplementation

E–H. Female Pdss2^kd/kd mice (100-day old) were fed standard diets plus (n = 3) or minus (n = 2) probucol for 15 days. Aliquots of kidney (E and F) and liver (G and H) homogenates were measured for content of total CoQ₉ (E and G) or total CoQ₁₀ (F and H). **p < 0.01. Both CoQ₉ and CoQ₉H₂ and CoQ₁₀ and CoQ₁₀H₂ were present in all extracts, with no differences between them as was observed in extracts prepared from the older mice (205–207 days of age) following long-term probucol treatment.