title: Alpha oxidation
text: # Alpha oxidation
  
  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 acetyl-CoA and subsequently become beta oxidized, yielding propionyl-CoA.
  
  ## Pathway
  
  Alpha-oxidation of phytanic acid is believed to take place entirely within peroxisomes.
  
  * Phytanic acid is first attached to CoA to form phytanoyl-CoA.
  * Phytanoyl-CoA is oxidized by phytanoyl-CoA dioxygenase, in a process using $Fe^{2+}$ and $O_2,$ to yield 2-hydroxyphytanoyl-CoA.
  * 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 $CO_2).$
  * 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.
  
  