Biochemical abnormalities in Huntington's chorea brains

Abstract

The enzymes glutamic acid decarboxylase, choline acetylase, and succinate dehydrogenase were reduced by 74 to 93 percent in striatum of brains from three patients who died with long-standing Huntington9s chorea. Little change was found in acetylcholinesterase or monoamine oxidase activities. In an early case of this disease in a fourth patient, there were no enzymatic changes. A high-molecular-weight soluble protein was present in the striatum of the advanced cases but membrane protein patterns did not differ from those of controls.