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少年型震颤麻痹(AR-JP)是一种常染色体隐性遗传病,多见于日本人群,大多数患者发病年龄<40岁,是左旋多巴有效的帕金森病(PD)。其病理改变为高选择性的黑质致密带多巴胺能神经元变性,伴有反应性神经胶质增生,但无Lewy体形成。锰-超氧化物歧化酶(MnSOD)是一种线粒体酶,可清除线粒体呼吸链产生的超氧阴离子。有研究显示,PD病人黑质中MnSOD活性增高,预示在PD病人脑中可能存在线粒体氧化反应。
Juvenile Parkinsonism (AR-JP) is an autosomal recessive disease more common in the Japanese population, most of whom are at onset age <40 years and are effective Parkinson’s disease (PD) with levodopa. The pathological changes of the highly selective substantia nigra compact dopaminergic neurons degeneration, accompanied by reactive glial proliferation, but no Lewy body formation. Manganese-superoxide dismutase (MnSOD) is a mitochondrial enzyme that clears superoxide anion produced by the mitochondrial respiratory chain. Studies have shown that increased activity of MnSOD in PD substantia nigra suggests that mitochondrial oxidation may exist in the brain of PD patients.