TABLE 1.

Plasma data at an age of 4 months of placebo mice or after 2 months of 4-PBA administration

Age of mice analyzed: 4 months. Data are the means ± S.D. n = 6–19 per genotype. P_i, inorganic phosphate; ALAT, alanine aminotransferase (EC 2.6.1.2); ASAT, aspartate aminotransferase (EC 2.6.1.1). 2-Way ANOVA: a, b, c, p < 0.05; aa, bb, cc, p < 0.01; aaa, bbb, ccc, p < 0.001; a, 4-PBA versus placebo of the same genotype; b, homozygous mutant versus wild type of the same treatment group; c, homozygous Umod^C93F mutant versus homozygous Umod^A227T mutant of the same treatment group.

Plasma parameters	Wild-type		UmodA227T homozygote		UmodC93F homozygote
	Placebo	4-PBA	Placebo	4-PBA	Placebo	4-PBA
Na+ (mmol/liter)	155.9 ± 3.1	154.3 ± 3.6	151.7 ± 6.6b	156.4 ± 2.3aa.b	155.5 ± 4.3c	156.1 ± 1.9
K+ (mmol/liter)	4.07 ± 0.29	3.94 ± 0.19	4.15 ± 0.40	4.21 ± 0.32bb	4.22 ± 0.59	3.98 ± 0.26
Cl− (mmol/liter)	114.6 ± 1.9	113.5 ± 2.8	111.8 ± 4.1b	114.5 ± 1.5a	113.9 ± 3.7	116.2 ± 1.1a,bb
Ca2+ (mmol/liter)	2.30 ± 0.07	2.21 ± 0.08a	2.32 ± 0.15	2.34 ± 0.09bbb	2.37 ± 0.07bbb	2.36 ± 0.11bbb
Pi (mmol/liter)	2.39 ± 0.44	2.11 ± 0.31	2.64 ± 0.70	1.94 ± 0.36aaa	2.30 ± 0.56	1.92 ± 0.26a
Total protein (g/liter)	51.1 ± 1.8	51.3 ± 2.7	49.6 ± 3.8	52.3 ± 1.7a	52.1 ± 2.4c	52.6 ± 1.7
Creatinine (μmol/liter)	9.00 ± 1.92	8.00 ± 1.32	9.86 ± 0.96	9.93 ± 1.30bbb	9.46 ± 1.83	9.45 ± 1.11b
Urea (mmol/liter)	10.15 ± 1.16	11.02 ± 1.29	20.54 ± 2.36bbb	20.49 ± 2.21bbb	23.08 ± 2.53bbb,c	25.99 ± 2.95aaa,bbb,ccc,
Cholesterol (mmol/liter)	3.56 ± 0.42	3.20 ± 0.42a	3.41 ± 0.16	3.31 ± 0.24	3.76 ± 0.25cc	3.45 ± 0.25a
Triglycerides (mmol/liter)	1.72 ± 0.87	1.67 ± 0.60	1.04 ± 0.27b	1.25 ± 0.51b	0.80 ± 0.24bbb	0.93 ± 0.23bb,cc
ALAT (units/liter)	32.1 ± 14.2	38.7 ± 24.0	29.3 ± 8.7	43.1 ± 44.8	28.1 ± 6.4	31.4 ± 5.2
ASAT (units/liter)	56.1 ± 14.5	68.7 ± 48.9	48.5 ± 5.4	72.3 ± 55.2	65.8 ± 29.5	58.3 ± 8.1
Lipase (units/liter)	45.6 ± 7.5	43.9 ± 7.8	54.2 ± 5.5bb	49.8 ± 8.2b	56.1 ± 6.0bbb	55.9 ± 4.7bbb,c