Import of Proteins Into Peroxisomes

Biochim Biophys Acta. 1999 Aug 12;1451(1):17-34. doi: 10.1016/s0167-4889(99)00087-7.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Intracellular Membranes / chemistry*
  • Membrane Proteins / chemistry
  • Microbodies / chemistry*
  • Peroxisomal Targeting Signal 2 Receptor
  • Peroxisome-Targeting Signal 1 Receptor
  • Protein Folding
  • Proteins / chemistry*
  • Receptors, Cytoplasmic and Nuclear / chemistry
  • Signal Transduction

Substances

  • Membrane Proteins
  • Peroxisomal Targeting Signal 2 Receptor
  • Peroxisome-Targeting Signal 1 Receptor
  • Proteins
  • Receptors, Cytoplasmic and Nuclear