. 2011 Jul;3(7):410–427. doi: 10.1002/emmm.201100149

Table 1.

Effects of probucol or CoQ₁₀ supplementation on renal disease manifestations of B6.Pdss2^kd/kd missense mutant mice

Strain	Gender	Treatment group	Treatment duration	N	Age at sacrifice (Days)	Nephritis score	Urine albumin (mg/24 h)	Albumin comparison group	Albumin p-value
B6	M	No treatment	n/a	14	156 ± 48.58	0.43 ± 0.46	0.46 ± 0.61
B6	F	No treatment	n/a	12	156 ± 59.57	0.63 ± 0.62	0.22 ± 0.12
B6.Pdss2^kd/kd	M	No treatment	n/a	24	135 ± 18.37	1.61 ± 0.93	10.60 ± 9.12
B6.Pdss2^kd/kd	F	No treatment	n/a	20	136 ± 20.49	2.69 ± 0.99	31.47 ± 30.12	B6.Pdss2^kd/kd males	p < 0.001
B6.Pdss2^kd/kd	M	CoQ10	Birth to sacrifice	7	145 ± 8.29	0.69 ± 0.43	3.64 ± 4.85	B6.Pdss2^kd/kd untreated males	p < 0.05
B6.Pdss2^kd/kd	F	CoQ10	Birth to sacrifice	13	144 ± 16.29	0.71 ± 0.70	2.24 ± 1.79	B6.Pdss2^kd/kd untreated females	p < 0.001
B6.Pdss2^kd/kd	M	CoQ10	Weaning to sacrifice	6	125 ± 4.5	1.67 ± 0.75	4.11 ± 2.04	B6.Pdss2^kd/kd untreated males	p < 0.01
B6.Pdss2^kd/kd	F	CoQ10	Weaning to sacrifice	4	124 ± 3.19	2.26 ± 0.92	1.75 ± 0.43	B6.Pdss2^kd/kd untreated females	p < 0.001
B6.Pdss2^kd/kd	M	CoQ10	Day 100 to sacrifice	6	121 ± 0.94	1.42 ± 0.45	10.47 ± 3.47	B6.Pdss2^kd/kd untreated males	p > 0.05
B6.Pdss2^kd/kd	F	CoQ10	Day 100 to sacrifice	5	121 ± 0	1.30 ± 0.40	4.08 ± 1.15	B6.Pdss2^kd/kd untreated females	p < 0.001
B6.Pdss2^kd/kd	M	Probucol	Birth to sacrifice^a	5	206 ± 0	0.6 ± 0.49	0.76 ± 0.14	B6.Pdss2^kd/kd untreated males	p < 0.001
B6.Pdss2^kd/kd	F	Probucol	Birth to sacrifice	3	206 ± 0	1.67 ± 0.47	19.01 ± 9.50	B6.Pdss2^kd/kd untreated females	p > 0.05
B6.Pdss2^kd/kd	M	Probucol	Weaning to sacrifice	14	156 ± 30.36	0.57 ± 0.82	1.15 ± 1.12	B6.Pdss2^kd/kd untreated males	p < 0.001
B6.Pdss2^kd/kd	F	Probucol	Weaning to sacrifice	11	136 ± 9.43	0.55 ± 0.99	1.42 ± 0.91	B6.Pdss2^kd/kd untreated females	p < 0.001
B6.Pdss2^kd/kd	M	Probucol	Day 100 to sacrifice	5	122 ± 0	1.40 ± 0.37	0.64 ± 0.17	B6.Pdss2^kd/kd untreated males	p < 0.001
B6.Pdss2^kd/kd	F	Probucol	Day 100 to sacrifice	4	122 ± 0	0.88 ± 0.55	1.53 ± 0.36	B6.Pdss2^kd/kd untreated females	p < 0.001
B6.Pdss2^kd/kd	M	Vitamin E	Weaning to sacrifice	9	122 ± 2.85	1.56 ± 0.50	11.58 ± 4.23	B6.Pdss2^kd/kd untreated males	p > 0.05
B6.Pdss2^kd/kd	F	Vitamin E	Weaning to sacrifice	10	120 ± 2.20	1.20 ± 0.40	11.38 ± 4.72	B6.Pdss2^kd/kd untreated females	p < 0.01

Probucol therapy significantly prevented renal sequelae that are typically manifest by 4 months of age whether administered either from the time of weaning or for only 3 weeks prior to sacrifice, with significant disease prevention still evident through approximately 6 months of age. CoQ₁₀ therapy significantly prevented renal manifestations only when provided from weaning through 4 months of age, although significantly greater effects were seen with probucol than CoQ₁₀ at all treatment durations tested (p < 0.01 at younger age when treated from weaning; p < 0.001 at younger age when treated for 3 weeks prior to sacrifice; and p < 0.001 at older age). All values are reported as mean ± standard deviation. p-Values were calculated based on urine albumin by Mann–Whitney U-test relative to designated (male or female) age-matched untreated B6.Pdss2^kd/kd animals.

Probucol treated missense mutant males showed the greatest therapeutic response in urine albumin at all ages examined, from the earliest age (122 days) through the oldest (206 days).