Patient | Control | |
---|---|---|
Enzyme activities (pmol/min/mg protein) | ||
ketoglutarate dehydrogenase (α-KGDH) | 900 | 7000 |
Isocitrate dehydrogenase (IDH) | 21000 | 23000 |
Pyruvate dehydrogenase (PDH) | 90 | 1117 |
Citrate Synthase (SC) | 27000 | 48000 |
Polarographic study (nmolO2/min/mg protein) | ||
Pyruvate + malate | 6.2 | 31.1 +/- 2 |
Malate + glutamate | 5.4 | 28.9 +/- 4.5 |
CO 2 production (nmol/h/mg of protein) | ||
Butyrate | 1.5 | 6 |
Octanoate | 0.9 | 3.4 |
Palmitate | 1.6 | 3.5 |
Glucose (1 mmol/l) | 2.8 | 7.8 |
Glucose (10 mmol/l) | 3.7 | 8.8 |
OH butyrate (1 mmol/l) | 0.8 | 3 |
OH butyrate (10 mmol/l) | 2.3 | 8.5 |