• lower trend in short-chain acyl-CoA levels than wild-type livers under both fed and fasted conditions
• modestly increased trend in short-chain acyl-CoA levels in the liver during a 48 h fast
• marked increase in long-chain acyl-CoAs in the livers after a 48 h fast
• significantly higher acetyl-carnitine levels in the livers in both the fed and fasted states
• striking accumulation of >C10 acyl-carnitines in both the fed and fasted states
• increased C16:0, C18:0 and C18:1 acyl-carnitines following a 48 h fast
• minor differences in the 3- and 4-carbon acyl-carnitines
• normal free carnitine level and normal number of mitochondria