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Biochimica et Biophysica Acta (BBA) - Molecular Cell Research
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Biochimica et Biophysica Acta (BBA) - Molecular Cell Research
Article . 2006 . Peer-reviewed
License: Elsevier Non-Commercial
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Peroxisomal disorders: The single peroxisomal enzyme deficiencies

Authors: Wanders, Ronald J.A.; Waterham, Hans R.;

Peroxisomal disorders: The single peroxisomal enzyme deficiencies

Abstract

Peroxisomal disorders are a group of inherited diseases in man in which either peroxisome biogenesis or one or more peroxisomal functions are impaired. The peroxisomal disorders identified to date are usually classified in two groups including: (1) the disorders of peroxisome biogenesis, and (2) the single peroxisomal enzyme deficiencies. This review is focused on the second group of disorders, which currently includes ten different diseases in which the mutant gene affects a protein involved in one of the following peroxisomal functions: (1) ether phospholipid (plasmalogen) biosynthesis; (2) fatty acid beta-oxidation; (3) peroxisomal alpha-oxidation; (4) glyoxylate detoxification, and (5) H2O2 metabolism.

Keywords

Hyperoxaluria, Alpha-oxidation, Fatty Acids, Plasmalogens, Beta-oxidation, Glyoxylates, Cell Biology, Hydrogen Peroxide, Plasmalogen, Peroxisomal Disorders, Refsum disease, Zellweger syndrome, Peroxisomes, Humans, Adrenoleukodystrophy, Molecular Biology, Oxidation-Reduction

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    selected citations
    These citations are derived from selected sources.
    This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
    250
    popularity
    This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
    Top 1%
    influence
    This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
    Top 1%
    impulse
    This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
    Top 1%
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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
250
Top 1%
Top 1%
Top 1%
hybrid