Table 3.

Probucol effects on plasma lipids in B6.Pdss2^kd/kd missense and B6.Podocin/cre,Pdss2^loxP/loxP conditional knockout mice

Mouse strain	N	Age (days)	Treatment	Cholesterol (mg/dl)	Triglycerides (mg/dl)	Urine albumin (mg/24 h)	Nephritis score (0–4)
B6	8	160 ± 13	Control	85.96 ± 17.0	75.94 ± 24.5	0.5 ± 0.1	0.07 ± 0.17
B6.Pdss2kd/kd	16	133 ± 7.2	Control	97.1 ± 24.8	67.0 ± 22.3	24.3 ± 17.2	3.4 ± 0.5
B6.Pdss2kd/kd	12	169 ± 23.4	Probucol	< 12.5a	42.7 ± 19.7	1.6 ± 1.0	1.0 ± 1.3
				(p < 0.001)	(p < 0.01)	(p < 0.001)	(p < 0.01)
Pod/cre	14	150 ± 9.8	Control	369.1 ± 168.4	114.5 ± 51.0	96.6 ± 31.1	3.7 ± 0.5
Pod/cre	11	164 ± 3.5	Probucol	<63.2 ± 68.9b	168.3 ± 77.4	45.6 ± 33.4	3.4 ± 0.7
				(p < 0.001)	(p = 0.069)	(p < 0.01)	(p = 0.178)

Probucol therapy significantly lowered plasma cholesterol and triglycerides in B6.Pdss2^kd/kd missense mutants, as well as plasma cholesterol in renal podocyte-conditional Pdss2 knockout mutants (‘pod/cre’). While renal disease manifestations of albuminuria and nephritis were prevented by probucol in missense mutants (consistent with data presented in Table 1), renal disease manifestations were largely unabated by probucol therapy in pod/cre conditional knockouts.

^aCholesterol levels in all samples from this group were below the level of detection.

^bCholesterol levels in four of the samples from this group were below the level of detection. All values are reported as mean ± standard deviation. p-Values were calculated by student's t-test for each mutation type relative to untreated mutant animals.