Alpha-oxidation

Alpha-oxidation of phytanic acid is believed to take place entirely within peroxisomes.

(Propionyl-CoA is released as a result of beta oxidation when the beta carbon is substituted)

Enzymatic deficiency in alpha-oxidation (most frequently in phytanoyl-CoA dioxygenase) leads to Refsum's disease, in which the accumulation of phytanic acid and its derivatives leads to neurological damage. Other disorders of peroxisome biogenesis also prevent alpha-oxidation from occurring.

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