Most fatty acids in the body contain
an even number of carbons. The normal end-products of
-oxidation of
these compounds are all acetyl-CoA.
-oxidation of
fatty acids containing an odd number of carbons yields
a propionyl-CoA in the last step. Propionyl-CoA cannot be used
directly in the citric acid cycle. Instead, it is converted, in
the following reactions (Figure 18.19),
to succinyl-CoA.
1. Propionyl-CoA + ATP + HCO3- <=> D-Methylmalonyl-CoA + ADP + Pi (catalyzed by Propionyl-CoA Carboxylase)
2. D-Methylmalonyl-CoA <=> L-Methylmalonyl-CoA (catalyzed by Methylmalonyl-CoA Epimerase)
3. D-Methylmalonyl-CoA <=> Succinyl-CoA (catalyzed by Methylmalonyl-CoA Mutase)
Methylmalonyl-CoA mutase, the catalyst for the third reaction in this process, requires vitamin B12 as a cofactor. The inability to catabolize propionyl-CoA properly has severe consequences in humans. Severe acidosis results, lowering blood pH and damaging the central nervous system.