Characterizing pathogenic processes in Batten disease: use of small eukaryotic model systems.
Characterizing pathogenic processes in Batten disease: use of small eukaryotic model systems.
复制标题
表征巴顿病的致病过程：使用小型真核模型系统。
DOI：
10.1016/j.bbadis.2006.08.010
复制
发表时间：
2006
期刊：
影响因子：
--
通讯作者：
Pearce,DavidA
中科院分区：
文献类型：
--
作者：
Phillips,SeassonN;Muzaffar,Neda;Codlin,Sandra;Korey,ChristopherA;Taschner,PeterEM;deVoer,Gert;Mole,SaraE;Pearce,DavidA
神经元蜡样脂褐质沉积症（NCL）是神经退行性疾病。尽管如此，小型模式生物，包括那些缺乏神经系统的生物，已经证明在研究疾病的机制和研究与每种疾病相关的保守蛋白质的功能方面是非常宝贵的。从单细胞酵母、酿酒酵母和粟酒裂殖酵母，到蠕虫、秀丽隐杆线虫和果蝇、黑腹果蝇，对这些生物体的生物化学，特别是遗传学研究为了解NCL提供了深入的见解。
The neuronal ceroid lipofuscinoses (NCLs) are neurodegenerative disorders. Nevertheless, small model organisms, including those lacking a nervous system, have proven invaluable in the study of mechanisms that underlie the disease and in studying the functions of the conserved proteins associated to each disease. From the single-celled yeast, Saccharomyces cerevisiae and Schizosaccharomyces pombe, to the worm, Caenorhabditis elegans and the fruitfly, Drosophila melanogaster, biochemical and, in particular, genetic studies on these organisms have provided insight into the NCLs.