TABLE 3:

Fibroblast biochemical analyses

PatientControlNormal Range
PDC activity, activated (in nmol/min/mg protein)0.142.451.26–4.42
PDC activity, inactivated (in nmol/min/mg protein)0.140.10.19–2.30
Pyruvate carboxylase activityNormal
PEP carboxylase activityNormal
PDC E3 activityNormal

  • Note.—PDC indicates pyruvate dehydrogenase complex; PEP, phosphoenol pyruvate.