Effect of pyridoxal 5-phosphate on the activity of GOT isozyme in plasma from patients with Duchenne muscular dystrophy.
GOT isozyme (soluble GOT, s-GOT and mitochondrial GOT, m-GOT) activities with and without reactivation by the co-enzyme pyridoxal 5-phosphate (PALP) in plasma from 42 patients with Duchenne muscular dystrophy (DMD), and 11 normal controls were investigated. The magnitude of the enzyme activities in patients at the early stage of DMD greatly exceeded those of controls. As the disease progressed, the levels gradually declined, but late stage values were still higher than the control values except s-GOT with PALP. At the mid stage as well as the late stage, the increase in m-GOT activity was approximately to double the unstimulated activity, indicating that, in these patients, as much as half the m-GOT in the circulation was in the inactive apoenzyme form, whereas s-GOT showed negligible increase following PALP addition at any stage.
D000293:Adolescent; D000328:Adult; D001219:Aspartate Aminotransferases / Q000097:blood*; D002648:Child; D006801:Humans; D007527:Isoenzymes / Q000097:blood*; D007700:Kinetics; D009136:Muscular Dystrophies / Q000201:enzymology*; D011732:Pyridoxal Phosphate / Q000494:pharmacology*
D007527:Isoenzymes; D011732:Pyridoxal Phosphate; D001219:Aspartate Aminotransferases