Department of Biochemistry and Cell Biology, Rice University, 6100 Main Street MS601, Houston, TX 77005, USA.
Traffic. 2012 Oct;13(10):1378-92. doi: 10.1111/j.1600-0854.2012.01393.x. Epub 2012 Jul 25.
Peroxisomes are ubiquitous organelles housing a variety of essential biochemical pathways. Peroxisome dysfunction causes a spectrum of human diseases known as peroxisome biogenesis disorders (PBD). Although much is known regarding the mechanism of peroxisome biogenesis, it is still unclear how peroxisome dysfunction leads to the disease state. Several recent studies have shown that mutations in Drosophila peroxin genes cause phenotypes similar to those seen in humans with PBDs suggesting that Drosophila might be a useful system to model PBDs. We have analyzed the proteome of Drosophila to identify the proteins involved in peroxisomal biogenesis and homeostasis as well as metabolic enzymes that function within the organelle. The subcellular localization of five of these predicted peroxisomal proteins was confirmed. Similar to Caenorhabditis elegans, Drosophila appears to only utilize the peroxisome targeting signal type 1 system for matrix protein import. This work will further our understanding of peroxisomes in Drosophila and add to the usefulness of this emerging model system.
过氧化物酶体是普遍存在的细胞器,其中包含多种重要的生化途径。过氧化物酶体功能障碍会导致一系列被称为过氧化物酶体生物发生障碍(PBD)的人类疾病。尽管人们已经了解了过氧化物酶体生物发生的机制,但仍不清楚过氧化物酶体功能障碍如何导致疾病状态。最近的几项研究表明,果蝇过氧化物酶基因的突变会导致与 PBD 患者相似的表型,这表明果蝇可能是一种有用的模型系统来模拟 PBD。我们已经分析了果蝇的蛋白质组,以鉴定参与过氧化物酶体生物发生和动态平衡以及在细胞器内发挥作用的代谢酶的蛋白质。其中五个预测的过氧化物酶体蛋白的亚细胞定位得到了证实。与秀丽隐杆线虫类似,果蝇似乎只利用过氧化物酶体靶向信号类型 1 系统进行基质蛋白的输入。这项工作将进一步加深我们对果蝇过氧化物酶体的理解,并增加这个新兴模型系统的实用性。