The genetics of peroxisome biogenesis

被引:87
作者
Sacksteder, KA [1 ]
Gould, SJ
机构
[1] Johns Hopkins Univ, Sch Med, Dept Biol Chem, Baltimore, MD 21205 USA
[2] Johns Hopkins Univ, Sch Med, Dept Cell Biol & Anat, Baltimore, MD 21205 USA
关键词
organelle assembly; peroxins; pex genes; protein import; Zellweger syndrome;
D O I
10.1146/annurev.genet.34.1.623
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
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.
引用
收藏
页码:623 / 652
页数:30
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