Acadl-SNP based genotyping assay for long-chain acyl-CoA dehydrogenase deficient mice

[Very-long-chain acyl-CoA dehydrogenase deficiency].

Fasting induces prominent proteomic changes in liver in very long chain Acyl-CoA dehydrogenase deficient mice

Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency (VLCADD) is a clinically heterogeneous disorder of mitochondrial fatty acid β-oxidation usually identified through newborn screening. Genotype-phenotype correlations have been defined, but considerable clinical heterogeneity still exists. Symptoms are often induced by physiological stress such as fasting or intercurrent illness, setting ...

Renal response to short- and long-term exercise in very-long-chain acyl-CoA dehydrogenase-deficient (VLCAD−/−) mice

BACKGROUND Deficiency of very long-chain acyl-CoA dehydrogenase (VLCAD) is the most common disorder of mitochondrial β-oxidation of long-chain fatty acids. In order to maintain glucose homeostasis, the kidney and liver as the main gluconeogenic organs play an important role under conditions of impaired fatty acid oxidation. However, little is known about how a defective fatty acid oxidation mac...

Medium-Chain Acyl-CoA Dehydrogenase Deficiency in Gene-Targeted Mice

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common inherited disorder of mitochondrial fatty acid beta-oxidation in humans. To better understand the pathogenesis of this disease, we developed a mouse model for MCAD deficiency (MCAD-/-) by gene targeting in embryonic stem (ES) cells. The MCAD-/- mice developed an organic aciduria and fatty liver, and showed profound cold in...

H2O2 release from the very long chain acyl-CoA dehydrogenase

Enhanced mitochondrial generation of oxidants, including hydrogen peroxide (H2O2), is related to a large number of pathological conditions, including diet-induced obesity and steatohepatosis. Indeed, we have previously shown that high fat diets increase the generation of H2O2 in liver mitochondria energized by activated fatty acids. Here, we further study fatty-acid induced H2O2 release in live...