Table 3 Evidence of mitochondrial dysfunction in genetic mouse models of FAOD
Enzyme deficiencyMitochondrial dysfunctionReferences
SCADMitochondrial swelling and microvesicular fatty changes in hepatocytes
Respiratory chain complex alterations
[92]
[94]
LCADLow concentrations of citric acid cycle intermediates[93]
VLCADAbnormal mitochondrial bioenergetics (uncoupled mitochondria, increase in glucose uptake and decrease in phosphocreatine/ATP ratio)
Induction of oxidative stress
[54,91]
[52]
MTP α subunit
LCHAD
Swelling and distortion of mitochondria
Induction of oxidative stress
[82]
[90]