Peroxisomes, lipid metabolism and lipotoxicity

Peroxisomes play an essential role in cellular lipid metabolism as exemplified by the existence of a number of genetic diseases in humans caused by the impaired function of one of the peroxisomal enzymes involved in lipid metabolism. Key pathways in which peroxisomes are involved include: (1.) fatty...

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Published inBiochimica et biophysica acta Vol. 1801; no. 3; pp. 272 - 280
Main Authors Wanders, R.J.A., Ferdinandusse, S., Brites, P., Kemp, S.
Format Journal Article
LanguageEnglish
Published Netherlands Elsevier B.V 01.03.2010
Subjects
Adrenoleukodystrophy
Animals
Bile acid
Disease Models, Animal
Fatty acid
Fatty acid oxidation
Fatty Acids - metabolism
Humans
Lipid Metabolism Disorders - metabolism
Lipid Peroxidation
Metabolic Networks and Pathways
Peroxisomes - metabolism
Plasmalogen
Refsum disease
Zellweger syndrome
Fatty acid oxidation
Adrenoleukodystrophy
Fatty acid
Bile acid
Plasmalogen
Zellweger syndrome
Refsum disease
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Summary:Peroxisomes play an essential role in cellular lipid metabolism as exemplified by the existence of a number of genetic diseases in humans caused by the impaired function of one of the peroxisomal enzymes involved in lipid metabolism. Key pathways in which peroxisomes are involved include: (1.) fatty acid beta-oxidation; (2.) etherphospholipid biosynthesis, and (3.) fatty acid alpha-oxidation. In this paper we will describe these different pathways in some detail and will provide an overview of peroxisomal disorders of metabolism and in addition discuss the toxicity of the intermediates of peroxisomal metabolism as they accumulate in the different peroxisomal deficiencies.
ISSN:1388-1981
0006-3002
1879-2618
DOI:10.1016/j.bbalip.2010.01.001