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Huntington舞蹈病其尾状核和壳核的小神经原明显减少,伴有星形细胞增生和大脑皮层的神经原的显著减少.死去的许多神经元可能是属于那些以γ-氨酪酸(GABA)为抑制性神经递质的细胞.生化分析发现Huntington舞蹈病患者的尾状核、壳核、苍白球和黑质的GABA明显减少而且合成GABA谷氨酸脱羧酶(GAD)的酶的活性也相应地降低.在纹状体GABA受体结合位点的密度正常,而毒蕈碱胆碱能结合位点的密度降低.其他与神经递质有关的生化改变是纹状体的合成乙酰胆碱的乙酰胆碱转氨酶的活性降低,苍白球和黑质的P物质减少.
Huntington’s disease significantly reduced the number of neurons in the caudate and putamen, with a marked decrease in astrocytosis and neurons in the cerebral cortex. Many of the neurons that died might belong to those with GABA, Is a neurotransmitter-inhibiting neurotransmitter. Biochemical analyzes revealed a significant reduction of GABA in the caudate, putamen, globus pallidus and substantia nigra of Huntington’s chorea and a corresponding increase in the activity of enzymes that synthesize GABA-glutamate decarboxylase (GAD) Density at the GABA receptor binding site of the striatum is normal, while the density of the muscarinic cholinergic binding site is reduced.Other biochemical changes associated with neurotransmitters are the acetylcholine transaminase Decreased activity of P globus pallidus and substantia nigra.