NORMAL GLUTAMIC-ACID DECARBOXYLASE ACTIVITY IN KIDNEY TISSUE FROM PATIENTS WITH HUNTINGTONS-DISEASE

This study demonstrated no deficiency of GAD [glutamic acid decarboxylase, EC 4.1.1.15] activity in kidney tissue from patients with Huntington''s Disease when compared to controls. There was a tendency for the values from the kidney tissue of the Huntington''s Disease patients to be higher than the control values but this was not significant (t test of means P > 0.2). The present finding of normal GAD activity in kidney from patients with Huntington''s Disease supports the hypothesis that a deficiency in the GAD/GABA system is not the basic biochemical defect produced by the Huntington''s gene. Although disordered brain GABA metabolism appears to be a consistent and important factor in Huntington''s Disease, its relationship to the underlying genetic defect remains to be elucidated.