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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.