I. J. van der Klei*

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapterAcademic


Peroxisomes are important cell organelles of a very simple architecture that display a wide repertoire of metaboLic functions. In man, over 50 peroxisomal enzymes are known. Major peroxisomal metaboLic pathways are α- and β-oxidation of fatty acids, ether Lipid and bile acid biosynthesis, and the metaboLism of glyoxylate, purines, polyamines, and d-amino acids. As yet, two types of peroxisomal disorders have been identified, namely, those in which the formation of the organelles is disturbed or in which a single peroxisomal enzyme or transporter is defective. Generally, in these patients, substrates that are degraded by peroxisomal enzymes accumulate, or compounds that are synthesized in these organelles are reduced. This causes a variety of cLinical symptoms, which are often very severe resulting in an early death.

Original languageEnglish
Title of host publicationPathobiology of Human Disease
Subtitle of host publicationA Dynamic Encyclopedia of Disease Mechanisms
EditorsL.M. McManus, R.N. Mitchell
Number of pages6
ISBN (Electronic)9780123864567
ISBN (Print)9780123864574
Publication statusPublished - 2014


  • Bile acid biosynthesis
  • Catalase
  • Ether Lipid biosynthesis
  • Hydrogen peroxide-producing oxidase
  • Microbody
  • Peroxin
  • Peroxisome
  • Peroxisome biogenesis disorder
  • PEX gene
  • Single peroxisomal enzyme deficiency
  • Α- and β-oxidation of fatty acids

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