Decreased Proline Endopeptidase Activity in the Basal Ganglia in Huntington's Disease

Abstract
Soluble proline endopeptidase (EC 3.4.21.26) activity was measured by a fluorometric assay in eight human brain areas (caudate nucleus, lateral globus pallidus, medial globus pallidus, substantia nigrazona compacta, substantia nigra‐zona reticulata, frontal cortex‐Brodmann area 10, temporal cortex‐Brodmann area 38, and hippocampus), in 10 control and 10 Huntington's disease brains. An abnormally low activity (22% of control activity) was found in the caudate nucleus of Huntington's disease brains; significantly decreased activity was also detected in the lateral globus pallidus and medial globus pallidus (37% and 40% of control, respectively).