Peroxisomes and Peroxisomal Disorders

  • Marjo S. van der Knaap
  • Jacob Valk

Abstract

Peroxisomes are organelles that are present in virtually all human cell types. Their diameter varies between 0.5 and 1.5 μm. Morphologically, peroxisomes are differentiated from mitochondria by their single membrane, electron-dense fine granular and homogeneous matrix and absence of cristae. They can also be differentiated from lysosomes, which often contain vacuoles and various inclusions, and have a positive acid phospatase reaction on histochemistry, whereas peroxisomes are positive in reactions which show the presence of catalase. The size and abundance of peroxisomes and their enzyme content vary considerably. A striking feature of peroxisomes is that their enzymes can be induced and that the whole organelle can proliferate in response to many stimuli including dietary, hormonal and other physiological changes. The most profound proliferations are caused by hypolipidemic drugs. In liver and kidney, peroxisomes are normally present in high numbers, whereas in skin fibroblasts relatively few peroxisomes are present. Peroxisomes are also numerous in cells specialized in lipid metabolism. In the nervous system peroxisomes are more abundant in the early postnatal period than in adults. Oligodendrocytes contain peroxisomes during the period of active myelination and the organelles can be demonstrated in the cellular processes that form the myelin sheath. Peroxisomes are required for the synthesis of essential myelin constituents.

Keywords

Respiration Lysine Prostaglandin Cytosol Catalase 

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Copyright information

© Springer-Verlag Berlin Heidelberg 1995

Authors and Affiliations

  • Marjo S. van der Knaap
    • 1
  • Jacob Valk
    • 2
  1. 1.Department of Child NeurologyFree University HospitalAmsterdamThe Netherlands
  2. 2.Department of Diagnostic RadiologyFree University HospitalAmsterdamThe Netherlands

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