Howard Hughes Medical Institute, University of Pennsylvania, 306 Leidy Laboratories, 433 South University Avenue, Philadelphia, Pennsylvania 19104, USA.
Nat Rev Genet. 2009 Jun;10(6):359-70. doi: 10.1038/nrg2563.
The fruitfly Drosophila melanogaster has enabled significant advances in neurodegenerative disease research, notably in the identification of genes that are required to maintain the structural integrity of the brain, defined by recessive mutations that cause adult onset neurodegeneration. Here, we survey these genes in the fly and classify them according to five key cell biological processes. Over half of these genes have counterparts in mice or humans that are also associated with neurodegeneration. Fly genetics continues to be instrumental in the analysis of degenerative disease, with notable recent advances in our understanding of several inherited disorders, Parkinson's disease, and the central role of mitochondria in neuronal maintenance.
黑腹果蝇(Drosophila melanogaster)在神经退行性疾病研究方面取得了重大进展,特别是在鉴定那些对于维持大脑结构完整性所必需的基因方面,这些基因是通过隐性突变导致成年后神经退行性变而确定的。在这里,我们对这些在果蝇中的基因进行了调查,并根据五个关键的细胞生物学过程对它们进行了分类。这些基因中有一半以上在老鼠或人类中也有对应物,这些对应物也与神经退行性变有关。果蝇遗传学在退行性疾病的分析中仍然具有重要意义,最近在我们对几种遗传性疾病、帕金森病以及线粒体在神经元维持中的核心作用的理解方面取得了显著进展。