Alpha oxidation

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title: "Alpha oxidation" type: doc version: 1 created: 2026-02-28 author: "Wikipedia contributors" status: active scope: public tags: ["biochemistry", "cell-biology", "lipid-metabolism", "metabolic-pathways", "fatty-acids"] description: "Metabolic pathway" topic_path: "science/biology" source: "https://en.wikipedia.org/wiki/Alpha_oxidation" license: "CC BY-SA 4.0" wikipedia_page_id: 0 wikipedia_revision_id: 0

::summary Metabolic pathway ::

Enzymatic steps of alpha oxidation|right|thumb|250px Alpha oxidation (α-oxidation) is a process by which certain branched-chain fatty acids are broken down by removal of a single carbon from the carboxyl end. In humans, alpha-oxidation is used in peroxisomes to break down dietary phytanic acid, which cannot undergo beta-oxidation due to its β-methyl branch, into pristanic acid. Pristanic acid can then acquire CoA and subsequently become beta oxidized, yielding propionyl-CoA.

Pathway

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

1. Phytanic acid is first attached to CoA to form phytanoyl-CoA.
2. Phytanoyl-CoA is oxidized by phytanoyl-CoA dioxygenase, in a process using Fe2+ and O2, to yield 2-hydroxyphytanoyl-CoA.
3. 2-hydroxyphytanoyl-CoA is cleaved by 2-hydroxyphytanoyl-CoA lyase in a TPP-dependent reaction to form pristanal and formyl-CoA (in turn later broken down into formate and eventually CO2).
4. Pristanal is oxidized by aldehyde dehydrogenase to form pristanic acid (which can then undergo beta-oxidation). (Propionyl-CoA is released as a result of beta oxidation when the beta carbon is substituted)

Deficiency

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.

References

References

1. (9 November 2010). "Fatty acid omega-oxidation as a rescue pathway for fatty acid oxidation disorders in humans: Fatty acid oxidation disorders". FEBS Journal.

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