The mitochondrial fatty acid beta-oxidation pathway

Schematic representation of the mitochondrial fatty acid beta-oxidation pathway. The pathway starts with the uptake of FAs and carnitine from the plasma compartment into the cell, followed by the transport of acyl-CoA esters into the mitochondria via the carnitine cycle and the actual stepwise degradation of acyl-CoAs via the beta-oxidation spiral with acetyl-CoA units as end product, which can either be converted into ketone bodies or combusted in the citric acid cycle to CO2 and H2O

Publication

The enzymology of mitochondrial fatty acid beta-oxidation and its application to follow-up analysis of positive neonatal screening results. (2010) Ronald J. A. Wanders, et al. J Inherit Metab Dis. 2010 Oct;33(5):479-494. Figure: F1. All organisms Bos taurus Sus scrofa Rattus norvegicus Homo sapiens Drosophila melanogaster

Disease mentions