The Biochemistry of AFLP: Fatty Acid Beta-Oxidation

source: http://lipidlibrary.aocs.org/animbio/fa-oxid/index.htm

As mentioned before, AFLP is thought to be caused by the deficiency of LCHAD (Long-Chain 3-hydroxyacyl-enzyme A dehydrogenase, or 3-hydroxy acyl CoA dehydrogenase as shown in the diagram) , an enzyme that is involved in the beta-oxidation of long-chain  fatty acids in the mitochondria.

Beta-Oxidation is the process of converting large fatty acid molecules into simpler, smaller molecules of acetyl CoA, with the release of FADH2 and NADH, which are used to produce ATP (the energy currency of the body) through the Electron Transport Chain.

When a pregnant woman suffers from AFLP, the enzyme LCHAD is deficient, or absent in the fetus. Thus, L-3-hydroxy acyl CoA cannot be converted to a smaller 3-ketoacyl CoA, which is simpler, smaller ketone body. Without this conversion, the L-3-hydroxy acyl CoA would accumulate in the liver, heart and kidneys, causing disruptions in their function and may lead to other liver function complications.






References:

Fillmore, N., Alrob, O. A., Lopaschuk, G. D., 2011. Fatty Acid β-Oxidation. [ONLINE] Available at: <http://lipidlibrary.aocs.org/animbio/fa-oxid/index.htm> [Accessed 17 January 2013]

Cardiomyopathies. 2012. Cardiomyopathies-Genetic. [ONLINE] Available at:http://neuromuscular.wustl.edu/msys/cardiac2.htm. [Accessed 15 January 2013].