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帕金森病的果蝇模型。

A Drosophila model of Parkinson's disease.

作者信息

Feany M B, Bender W W

机构信息

Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.

出版信息

Nature. 2000 Mar 23;404(6776):394-8. doi: 10.1038/35006074.

Abstract

Parkinson's disease is a common neurodegenerative syndrome characterized by loss of dopaminergic neurons in the substantia nigra, formation of filamentous intraneuronal inclusions (Lewy bodies) and an extrapyramidal movement disorder. Mutations in the alpha-synuclein gene are linked to familial Parkinson's disease and alpha-synuclein accumulates in Lewy bodies and Lewy neurites. Here we express normal and mutant forms of alpha-synuclein in Drosophila and produce adult-onset loss of dopaminergic neurons, filamentous intraneuronal inclusions containing alpha-synuclein and locomotor dysfunction. Our Drosophila model thus recapitulates the essential features of the human disorder, and makes possible a powerful genetic approach to Parkinson's disease.

摘要

帕金森病是一种常见的神经退行性综合征,其特征为黑质中多巴胺能神经元丧失、神经元内丝状包涵体(路易小体)形成以及锥体外系运动障碍。α-突触核蛋白基因突变与家族性帕金森病相关,且α-突触核蛋白在路易小体和路易神经突中积累。在此,我们在果蝇中表达正常和突变形式的α-突触核蛋白,导致成年期多巴胺能神经元丧失、含有α-突触核蛋白的丝状神经元内包涵体形成以及运动功能障碍。因此,我们的果蝇模型概括了人类疾病的基本特征,并为帕金森病研究提供了一种强大的遗传学方法。

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