Add to ORKGIn this paper, we present a new method for measurement of long-chain acyl-CoA dehydrogenase (LCAD) activities in cultured skin fibroblasts. The method is based upon gas chromatographic/mass spectrometric determination of 3-OH-hexadecanoic acid formed during incubation of fibroblasts in a medium containing palmitoyl-CoA and crotonase, to convert the enoyl-CoA ester produced into the 3-hydroxyacyl-CoA ester. The validity of the method is demonstrated by the finding of a full deficiency of LCAD in fibroblasts from three patients with an established deficiency of LCAD.</p
Medium-chain fatty acids (mc-FAs) are currently applied in the treatment of long-chain fatty acid ox...
Background: Acyl-CoA thioesterases are enzymes that hydrolyze acyl-CoAs to the free fatty acid and c...
Background: Disorders of fatty acid oxidation (FAO) are difficult to diagnose, primarily because in ...
In this paper, we present a new method for measurement of long-chain acyl-CoA dehydrogenase (LCAD) a...
Short-chain acyl-CoA dehydrogenase (SCAD) deficiency has so far been reported in only very few patie...
Medium chain acyl-CoA dehydrogenase deficiency, a defect of mitochondrial beta-oxidation, is one of ...
International audienceMitochondrial fatty acid oxidation is a vital biochemical process for energy m...
In relation to the finding that human skin fibroblasts are capable of de novo ether phospholipid bio...
In relation to the finding that human skin fibroblasts are capable of de novo either phospholipid bi...
Inborn defects in mitochondrial B-oxidation of fatty acids are a group of diseases affecting humans ...
BACKGROUND: Two separate and complementary assays, total mitochondrial fatty acid beta-oxidation (FA...
The aim of this work was to establish an isolation method for recombinantly expressed human Acyl-CoA...
Newborn screening for medium- and very long-chain acyl-CoA dehydrogenase (MCAD and VLCAD, respective...
BACKGROUND:Newborn screening for medium- and very long-chain acyl-CoA dehydrogenase (MCAD and VLCAD,...
We describe a method for the diagnosis of mitochondrial fatty acid oxidation disorders that is based...
Medium-chain fatty acids (mc-FAs) are currently applied in the treatment of long-chain fatty acid ox...
Background: Acyl-CoA thioesterases are enzymes that hydrolyze acyl-CoAs to the free fatty acid and c...
Background: Disorders of fatty acid oxidation (FAO) are difficult to diagnose, primarily because in ...
In this paper, we present a new method for measurement of long-chain acyl-CoA dehydrogenase (LCAD) a...
Short-chain acyl-CoA dehydrogenase (SCAD) deficiency has so far been reported in only very few patie...
Medium chain acyl-CoA dehydrogenase deficiency, a defect of mitochondrial beta-oxidation, is one of ...
International audienceMitochondrial fatty acid oxidation is a vital biochemical process for energy m...
In relation to the finding that human skin fibroblasts are capable of de novo ether phospholipid bio...
In relation to the finding that human skin fibroblasts are capable of de novo either phospholipid bi...
Inborn defects in mitochondrial B-oxidation of fatty acids are a group of diseases affecting humans ...
BACKGROUND: Two separate and complementary assays, total mitochondrial fatty acid beta-oxidation (FA...
The aim of this work was to establish an isolation method for recombinantly expressed human Acyl-CoA...
Newborn screening for medium- and very long-chain acyl-CoA dehydrogenase (MCAD and VLCAD, respective...
BACKGROUND:Newborn screening for medium- and very long-chain acyl-CoA dehydrogenase (MCAD and VLCAD,...
We describe a method for the diagnosis of mitochondrial fatty acid oxidation disorders that is based...
Medium-chain fatty acids (mc-FAs) are currently applied in the treatment of long-chain fatty acid ox...
Background: Acyl-CoA thioesterases are enzymes that hydrolyze acyl-CoAs to the free fatty acid and c...
Background: Disorders of fatty acid oxidation (FAO) are difficult to diagnose, primarily because in ...