Decreased proline endopeptidase activity in the basal ganglia in Huntington's disease.
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 nigra-zona 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).
D001479:Basal Ganglia / Q000201:enzymology*; D001921:Brain / Q000201:enzymology*; D002421:Caudate Nucleus / Q000201:enzymology; D010450:Endopeptidases / Q000378:metabolism*; D006624:Hippocampus / Q000201:enzymology; D006801:Humans; D006816:Huntington Disease / Q000201:enzymology*; D007700:Kinetics; D009928:Organ Specificity; D000085822:Prolyl Oligopeptidases; D012016:Reference Values; D012697:Serine Endopeptidases
D010450:Endopeptidases; D012697:Serine Endopeptidases; C000705287:PREPL protein, human; D000085822:Prolyl Oligopeptidases