D-Hydroxyacyl-CoA Dehydrogenase Deficiency
Current Views of Fatty Acid Oxidation and KetogenesisAdvances in Experimental Medicine and Biology(2002)
Abstract
The second and third steps of peroxisomal β-oxidation are catalysed by two multifunctional enzymes: D-bifunctional protein and L-bifunctional protein. Here we show that fibroblasts of a patient described as being deficient in the 3-hydroxyacyl-CoA dehydrogenase component of D-bifunctional protein and fibroblasts of a patient described as being deficient in L-bifunctional protein do not complement one another. Using a newly developed method to measure the activity of D-bifunctional protein in fibroblast homogenates, we found that the activity of the D-bifunctional protein was completely deficient in the patient with presumed L-bifunctional protein deficiency.
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