Metabolic steps | Substrates | Patient | Control tested in the same run | Controls(n = 40) |
---|---|---|---|---|
(nmol/hr.mg protein) | (nmol/hr.mg protein) | (nmol/hr.mg protein) | ||
Mitochondrial oxidations assayed | ||||
- by carbon dioxide production | [1-14C]-butyrate | 2.61 | 6.60 | 4.20 ± 1.06[2.52-7.69] |
[1-14C]-octanoate | 1.41 | 4.77 | 3.53 ± 0.81[3.53-6.37] | |
- by carbon dioxide plus water soluble material production | [1-14C]-palmitate | 5.70 | 7.39 | 6.08 ± 1.03[4.28-8.51] |
- by tritiated water formation | [9,10-3H]-myristic acid | 1.03 | 9.27 | 7.75 ± 1.73[4.97-11.20] |
[9,10-3H]-palmitic acid | 1.75 | 7.41 | 6.12 ± 1.28[4.00-9.20] |