TABLE 1.
Characteristics of lysosomal acid lipase-deficient female mice at different stages of disease progression.
Data are presented as mean ± S.E.M. of values in four mice per group. All mice were weaned at 21 days of age and thereafter fed a basal rodent chow diet ad libitum. They were studied in the fed state.
Age |
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21 days |
49–51 days |
140–142 days |
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Lal+/+ | Lal−/− | Lal+/+ | Lal−/− | Lal+/+ | Lal−/− | |
Body weight (g) | 10.6 ± 0.6 | 10.3 ± 0.4 | 20.8 ± 0.3 | 20.8 ± 0.5 | 26.1 ± 0.5 | 24.2 ± 1.1 |
Liver weight (g) | 0.53 ± 0.04 | 0.64 ± 0.02 | 1.07 ± 0.04 | 2.32 ± 0.10* | 1.08 ± 0.04 | 4.93 ± 0.26* |
Hepatic esterified cholesterol concentration (mg/g) | 1.0 ± 0.3 | 30.0 ± 0.6* | 0.4 ± 0.1 | 56.3 ± 3.8* | 0.8 ± 0.1 | 53.7 ± 2.3* |
Hepatic unesterified cholesterol concentration (mg/g) | 2.1 ± 0.1 | 2.7 ± 0.1* | 2.0 ± 0.1 | 3.2 ± 0.1* | 2.0 ± 0.1 | 6.8 ± 0.5* |
Whole-liver cholesterol content (mg/organ) | 1.7 ± 0.2 | 20.8 ± 1.0* | 2.6 ± 0.1 | 138.1 ± 11.3* | 3.0 ± 0.2 | 297 ± 10.7* |
Hepatic triacylglycerol concentration (mg/g) | 7.2 ± 2.0 | 10.2 ± 1.8 | 6.0 ± 0.7 | 14.6 ± 0.6* | 12.6 ± 1.5 | 19.4 ± 0.8* |
Whole-liver triacylglycerol content (mg/organ) | 3.9 ± 1.1 | 6.5 ± 1.3 | 6.5 ± 0.8 | 40.0 ± 2.8* | 13.6 ± 1.9 | 97.4 ± 10.6* |
Plasma ALT activity (units/l) | 32 ± 2 | 39 ± 5 | 33 ± 2 | 268 ± 37* | 28 ± 4 | 617 ± 97* |
P < 0.05 compared with corresponding Lal+/+ mice of the same age.