Import of proteins into peroxisomes.
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.
Hettema
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EH
Department of Biochemistry, Academic Medical Centre, Meibergdreef 15, 1105 AZ, Amsterdam, Netherlands.
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D007425:Intracellular Membranes / Q000737:chemistry*; D008565:Membrane Proteins / Q000737:chemistry; D008830:Microbodies / Q000737:chemistry*; D000074437:Peroxisomal Targeting Signal 2 Receptor; D000074435:Peroxisome-Targeting Signal 1 Receptor; D017510:Protein Folding; D011506:Proteins / Q000737:chemistry*; D018160:Receptors, Cytoplasmic and Nuclear / Q000737:chemistry; D015398:Signal Transduction
D008565:Membrane Proteins; D000074437:Peroxisomal Targeting Signal 2 Receptor; D000074435:Peroxisome-Targeting Signal 1 Receptor; D011506:Proteins; D018160:Receptors, Cytoplasmic and Nuclear