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Updated: Aug 10 2019

Propionic Acid Pathway

  • Overview
    • Production
      • β-oxidation of odd-numbered fatty acids
        • 2 carbon acetyl-CoA groups removed from chain until there are 5 carbons remaining
        • 5 carbon chain split into 1 acetyl-CoA (2C) + 1 propionyl-CoA (3C)
    • Pathway
      • propionyl-CoA → methylmalonyl-CoA
        • catalyzed by propionyl-CoA carboxylase
          • requires biotin (B7)
      • methylmalonyl-CoA → succinyl-CoA
        • catalyzed by methylmalonyl-CoA mutase
          • requires B12
        • deficiency of B12 results in a blockage of this step
          • result is ↑↑↑ methylmalonate
          • can cause irreversible neuropathy
            • due to pathologic synthesis of myelin with methylmalonate
          • a means to determine whether a patient with megaloblastic anemia is deficient in folate or B12
            • methylmalonic aciduria not see in folate deficiencies
      • succinyl-CoA → citric acid cycle
        • can be converted to malate
          • is gluconeogenic
            • exception to rule that fatty acids can be converted to glucose
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