Abstract
In a patient with vitamin B6 dependent convulsions, the activity of glutamie acid decarboxylase (GAD) was found to be decreased when pyridoxal-phosphate (PALP) was not added in the reaction system for the enzyme assay. The activity was restored to normal level when PALP was added in large amounts in the reaction system. These findings suggest that the metabolic abnormality in this disorder exists in the binding abnormality between GAD apoenzyme and PALP.