Alpha oxidation

Alpha oxidation (α-oxidation) is a process by which certain branched-chain[1] fatty acids are broken down by removal of a single carbon from the carboxyl end.

Pristanic acid can then acquire CoA and subsequently become beta oxidized, yielding propionyl-CoA.

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.

Enzymatic steps of alpha oxidation