TY - JOUR
T1 - 3-Hydroxy-fatty acid analysis by gas chromatography-mass spectrometry.
AU - Jones, Patricia M.
AU - Bennett, Michael J.
PY - 2010
Y1 - 2010
N2 - The mitochondrial fatty acid beta-oxidation is integral to normal cellular metabolism and maintenance of cellular energy supplies. Disorders of this pathway interrupt the body's ability to deal with fasting states, as well as compromising the functioning of organs and systems whose high-energy requirements utilize fats for a continuous energy source, such as heart and skeletal muscle. This method quantitatively measures intermediate metabolites of fatty acid beta-oxidation, specifically the 3-hydroxy-fatty acids produced by the third step in the pathway. The method is useful for helping to diagnose disorders of the pathway, especially defects in the L-3-hydroxyacyl CoA dehydrogenases. Serum or plasma samples are used for routine clinical evaluation; however, measurement of 3-hydroxy-fatty acid intermediates in fibroblast cell culture media and in samples from mice also allows the method to be used for research into fatty acid oxidation and interconnected pathways. The method is a stable isotope dilution, electron impact ionization gas chromatography/mass spectrometry (GC/MS) procedure.
AB - The mitochondrial fatty acid beta-oxidation is integral to normal cellular metabolism and maintenance of cellular energy supplies. Disorders of this pathway interrupt the body's ability to deal with fasting states, as well as compromising the functioning of organs and systems whose high-energy requirements utilize fats for a continuous energy source, such as heart and skeletal muscle. This method quantitatively measures intermediate metabolites of fatty acid beta-oxidation, specifically the 3-hydroxy-fatty acids produced by the third step in the pathway. The method is useful for helping to diagnose disorders of the pathway, especially defects in the L-3-hydroxyacyl CoA dehydrogenases. Serum or plasma samples are used for routine clinical evaluation; however, measurement of 3-hydroxy-fatty acid intermediates in fibroblast cell culture media and in samples from mice also allows the method to be used for research into fatty acid oxidation and interconnected pathways. The method is a stable isotope dilution, electron impact ionization gas chromatography/mass spectrometry (GC/MS) procedure.
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U2 - 10.1007/978-1-60761-459-3_21
DO - 10.1007/978-1-60761-459-3_21
M3 - Article
C2 - 20077074
AN - SCOPUS:77950362654
SN - 1064-3745
VL - 603
SP - 229
EP - 243
JO - Methods in molecular biology (Clifton, N.J.)
JF - Methods in molecular biology (Clifton, N.J.)
ER -