Other Oxidations

of Fatty Acid
 Types
• β-oxidation

• α-oxidation

• ω-oxidation
 α-oxidation
• Process by which certain fatty acids are
broken down by removal of a single carbon
from the carboxyl end of a fatty acid

• Removal of One Carbon unit at a time by the

oxidation of alpha carbon of fatty acid
 Contd.
• Some Fatty Acids cannot undergo Beta oxidation
due to Beta-Methyl branch on its 3rd (beta)
Carbon.

• Oxidation occurs at Alpha Carbon.

• Does not involve binding of fatty acid to Acyl CoA

dehydrogenase

• No energy is produced.
• Phytanic acid cannot undergo Beta oxidation

• It is converted to Pristanic Acid (undergoes B-

oxidation) by removal of one Carbon

• The process occurs in Peroxisomes

 Phytanic Acid

• Derived from Phytol, a constituent of

Chlorophyll.
• Found mostly in plant foods
• Also present in milk lipids and animal fats
• Methyl group is present on Carbon 3
• Undergoes initial alpha oxidation; removal of
alpha carbon as CO2
• Followed by Beta oxidation
Alpha Oxidation Pathway
 Steps of Alpha Oxidation Pathway
• Phytanic is first attached to CoA to form
Phytonyl-CoA
• Phytonyl-CoA is oxidized by Phytonyl-CoA
dioxygenase to yield 2-hydroxy-phytonyl CoA.
• 2-hydroxy-phytonyl-CoA is cleaved by Lyase to
form Pristanal and formyl CoA
• Pristanal is Oxidized by aldehyde
dehydrogenase to form Pristanic Acid.
Clinical Abnormality Due to defective
Alpha Oxidation
• Refsum’s Disease
• Due to deficiency of Phytonyl-CoA
dioxygenase (phytanic acid alpha oxidase)
• Accumulation of Phytanic acid and its
derivatives
• Characterized by Cerebral ataxia and
Peripheral Neuropathy.
 SYMPTOMS OF
REFSUM’S DISEASE
• Scaly Skin
• Night Blindness
•Ataxia
•Hearing Problem
•Cataracts
•Elevation of Protein in CSF
•Shortening of fourth toe
Characteristic Shortening of fourth toe
in Refsum’s disease
 Treatment

• Avoid the consumption of green leafy

Vegetables

•Phytanic Acid-restricted diet

• Plasmapheresis
 ω oxidation
• Involves the oxidation of ω-Carbon (the
carbon most distant from the carboxyl group
of fatty acid)

• Minor catabolic Pathway for MCFA

• Becomes more important when Beta

Oxidation is defective
 Contd.
• Enzymes located in ER of Liver or Kidney

• It involves Hydroxylation and Oxidation steps

•Hydroxylation Step requires Cytochrome P450

•After Oxidation, either side of FA can combine with

CoA and undergo Beta Oxidation

•Succinic Acid; Final product. Enters Citric Acid

Cycle