The genetics of peroxisome biogenesis

K. A. Sacksteder, S. J. Gould

Research output: Contribution to journalReview articlepeer-review

96 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)623-652
Number of pages30
JournalAnnual review of genetics
StatePublished - Dec 1 2000


  • Organelle assembly
  • Peroxins
  • Protein import
  • Zellweger syndrome
  • pex genes

ASJC Scopus subject areas

  • Genetics


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