Sacksteder K A, Gould S J
Department of Biological Chemistry, the Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Annu Rev Genet. 2000;34:623-652. doi: 10.1146/annurev.genet.34.1.623.
The segregation of metabolic functions within discrete organelles is a hallmark of eukaryotic cells. These compartments allow for the concentration of related metabolic functions, the separation of competing metabolic functions, and the formation of unique chemical microenvironments. However, such organization is not spontaneous and requires an array of genes that are dedicated to the assembly and maintenance of these structures. In this review we focus on the genetics of peroxisome biogenesis and on how defects in this process cause human disease.
代谢功能在离散细胞器内的分隔是真核细胞的一个标志。这些区室允许相关代谢功能的集中、竞争性代谢功能的分离以及独特化学微环境的形成。然而，这种组织并非自发形成，需要一系列专门用于这些结构组装和维持的基因。在本综述中，我们重点关注过氧化物酶体生物发生的遗传学以及该过程中的缺陷如何导致人类疾病。
