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Disorders of peroxisomal metabolism in adults 

Disorders of peroxisomal metabolism in adults
Chapter:
Disorders of peroxisomal metabolism in adults
Author(s):

Anthony S. Wierzbicki

DOI:
10.1093/med/9780199204854.003.1209_update_002

July 30, 2015: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

Update:

Data on biochemistry of peroxisomal enzymes and transporters updated.

Mechanism of toxicity of phytanic and pristanic acids updated.

Description of the adult presentation of acyl-coenzymeA oxidase deficiency as the cause of pseudoadrenoleukodystrophy.

Data on the pathophysiological mechanisms behind adrenoleukodystrophy updated.

Updated on 28 Nov 2013. The previous version of this content can be found here.
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date: 21 October 2017

The peroxisome is a specialized organelle which employs molecular oxygen in the oxidation of complex organic molecules including lipids. Enzymatic pathways for the metabolism of fatty acids, including very long-chain fatty acids (VLCFA) enable this organelle to carry out β-oxidation in partnership with mitochondria. A peroxisomal pathway for isoprenoid lipids derived from chlorophyll, such as phytanic acid, utilizes ...

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