亨廷顿舞蹈病患者基底神经节中脯氨酸内肽酶活性降低。

Decreased proline endopeptidase activity in the basal ganglia in Huntington's disease.

作者信息

Pittaway K M, Reynolds G P, Emson P C

出版信息

J Neurochem. 1984 Sep;43(3):878-80. doi: 10.1111/j.1471-4159.1984.tb12813.x.

DOI:10.1111/j.1471-4159.1984.tb12813.x

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 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).

摘要

采用荧光分析法，在10例对照者和10例亨廷顿舞蹈病患者的8个脑区（尾状核、外侧苍白球、内侧苍白球、黑质致密部、黑质网状部、额叶皮质-布罗德曼第10区、颞叶皮质-布罗德曼第38区和海马体）中测定了可溶性脯氨酸内肽酶（EC 3.4.21.26）的活性。在亨廷顿舞蹈病患者的尾状核中发现活性异常低（为对照活性的22%）；在外侧苍白球和内侧苍白球中也检测到活性显著降低（分别为对照的37%和40%）。