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Disorders of peroxisomal metabolism in adults 

Disorders of peroxisomal metabolism in adults
Disorders of peroxisomal metabolism in adults

Anthony S. Wierzbicki


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


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.

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date: 01 December 2021

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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