Measurement of tissue acyl-CoAs using flow-injection tandem mass spectrometry: acyl-CoA profiles in short-chain fatty acid oxidation defects

AA Palladino, J Chen, S Kallish, CA Stanley… - Molecular genetics and …, 2012 - Elsevier
The primary accumulating metabolites in fatty acid oxidation defects are intramitochondrial
acyl-CoAs. Typically, secondary metabolites such as acylcarnitines, acylglycines and
dicarboxylic acids are measured to study these disorders. Methods have not been adapted
for tissue acyl-CoA measurement in defects with primarily acyl-CoA accumulation. Our
objective was to develop a method to measure fatty acyl-CoA species that are present in
tissues of mice with fatty acid oxidation defects using flow-injection tandem mass …