Hettema E H, Distel B, Tabak H F
Department of Biochemistry, Academic Medical Centre, Meibergdreef 15, 1105 AZ, Amsterdam, Netherlands.
Biochim Biophys Acta. 1999 Aug 12;1451(1):17-34. doi: 10.1016/s0167-4889(99)00087-7.
Peroxisomes are organelles that confine an important set of enzymes within their single membrane boundaries. In man, a wide variety of genetic disorders is caused by loss of peroxisome function. In the most severe cases, the clinical phenotype indicates that abnormalities begin to appear during embryological development. In less severe cases, the quality of life of adults is affected. Research on yeast model systems has contributed to a better understanding of peroxisome formation and maintenance. This framework of knowledge has made it possible to understand the molecular basis of most of the peroxisome biogenesis disorders. Interestingly, most peroxisome biogenesis disorders are caused by a failure to target peroxisomal proteins to the organellar matrix or membrane, which classifies them as protein targeting diseases. Here we review recent fundamental research on peroxisomal protein targeting and discuss a few burning questions in the field concerning the origin of peroxisomes.
过氧化物酶体是一种细胞器，它将一组重要的酶限制在其单一膜边界内。在人类中，多种遗传疾病是由过氧化物酶体功能丧失引起的。在最严重的情况下，临床表型表明异常在胚胎发育过程中就开始出现。在不太严重的情况下，成年人的生活质量会受到影响。对酵母模型系统的研究有助于更好地理解过氧化物酶体的形成和维持。这一知识框架使得理解大多数过氧化物酶体生物发生障碍的分子基础成为可能。有趣的是，大多数过氧化物酶体生物发生障碍是由于过氧化物酶体蛋白无法靶向到细胞器基质或膜上所致，这将它们归类为蛋白质靶向疾病。在此，我们综述了关于过氧化物酶体蛋白靶向的最新基础研究，并讨论了该领域中一些关于过氧化物酶体起源的紧迫问题。
