toxicity of 3-hydroxylated fatty acids

Posted by & filed under _.

Cardiomyopathy is a well known complication of LCHAD deficiency and Mitochondrial Trifunctional protein (MTP) deficiency. Tonin et al (in J Bioenerg Biomech 2013 Feb;45(1-2):47-57) offer evidence that several 3-hydroxylated fatty acids that accumulate in MTP and LCHAD deficiencies act as uncouplers of oxidative phosphorylation.This could potentially interfere with cardiac energy homeostasis. It would be interesting to better understand the accumulation of these potentially toxic 3-hydroxylated fatty acids during intercurrent illness, and see if these measurements could predict possibility of cardiac decompensation.

Hilary Vernon MD PhD

Leave a Reply

You must be logged in to post a comment.