TABLE 3

Function of mouse liver mitochondria

The mice were starved overnight before the final experiments (n = 5 for each group). Wild-type mice and jvs+/- mice were given oral treatments with normal saline or VPA for 14 days. Mitochondria were isolated by differential centrifugation. State 3 oxidation rates were determined using different substrates, and the in vitro β-oxidation was measured with [1-14C]palmitic acid. Units are atoms of oxygen per minute per milligram of mitochondrial protein for the oxidation rates, and nanomoles per minute per milligram of mitochondrial protein for β-oxidation. Results are presented as the mean ± S.D.

Wild-Type (jvs+/+)
Heterozygous (jvs+/-)
Vehicle
VPA
Vehicle
VPA
State 3 oxidation rates
    l-Glutamate (20 mM) 56 ± 12 38 ± 8* 48 ± 8 28 ± 2†
    Succinate (20 mM) 120 ± 20 88 ± 28 122 ± 28 64 ± 10†
    Palmitoyl-CoA (40 μM) 42 ± 12 24 ± 8* 30 ± 8 8 ± 2†‡
Mitochondrial β-oxidation
    β-Oxidation of [1-14C]palmitic palmitate
0.27 ± 0.04
0.15 ± 0.02*
0.26 ± 0.04
0.17 ± 0.04†

  • ↵* P < 0.05, vehicle-treated jvs+/- mice or VPA-treated wild-type mice vs. vehicle-treated wild-type mice.

  • ↵† P < 0.05, VPA-treated jvs+/- mice vs. vehicle-treated jvs+/- mice.

  • ↵‡ P < 0.05, VPA-treated jvs+/- mice vs. VPA-treated wild-type mice.