Principles of Biochemistry, Volume 2 - A. Lehninger 1985

Bioenergetics and Metabolism
Fatty Acid Oxidation in Animal Tissues
Hypoglycin (a toxic substance produced by certain plants) inhibits fatty acid oxidation

Among the impoverished population of Jamaica, there is an endemic disease that has long been known to be associated with the consumption of unripe fruit of Blighia sapida. This condition is characterized by hypoglycemia (low Blood sugar) and impaired fatty acid METABOLISM. The Toxic Effect of Blighia sapida fruit is caused by hypoglycin, a propionic acid derivative contained within it (Fig. 18-13). During metabolism, hypoglycin is converted into a substance that, in the form of its corresponding CoA ester, acts as a potent and specific inhibitor of The oxidation of short-chain fatty acyl-CoA esters, primarily butyryl-CoA. In the presence of this substance, butyryl-CoA is hydrolyzed to form free butyrate, which accumulates in excess in the blood and indirectly induces hypoglycemia.

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Fig. 18-13. Hypoglycin A, found in unripe fruits of Blighia sapida, is enzymatically converted into an exceptionally potent inhibitor of the oxidation of short-chain fatty acyl-CoA esters.



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