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Peroxisomes, lipid metabolism, and peroxisomal disorders

Journal

MOLECULAR GENETICS AND METABOLISM
Volume 83, Issue 1-2, Pages 16-27

Publisher

ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.ymgme.2004.08.016

Keywords

etherphospholipid biosynthesis; fatty acid beta-oxidation; fatty acid alpha-oxidation; peroxisome biogenesis; Zellweger syndrome; Refsum disease

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Peroxisomes catalyse a large variety of different cellular functions of which most have to do with lipid metabolism. This paper deals with the role of peroxisomes in three key pathways of lipid metabolism, including: (1) etherphospholipid biosynthesis, (2) fatty acid beta-oxidation, and (3) fatty acid alpha-oxidation. Apart from a brief description of the peroxisomal enzymes involved in each of these pathways, the interaction between peroxisomes and other subcellular organelles, notably microsomes and peroxisomes, will be discussed. Finally, the current state of knowledge with respect to the different disorders of peroxisomal lipid metabolism will be described. (C) 2004 Elsevier Inc. All rights reserved.

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