Long-chain fatty acid beta-oxidation disorders (lcFAOD) are a group of inherited metabolic diseases, characterized by the accumulation of disease-specific acyl-CoAs reflecting the enzyme deficiency.

Carnitine palmitoyltransferase II (CPT2) deficiency is a rare autosomal recessive disorder of mitochondrial fatty acid oxidation. The most common form of this disorder is characterized by muscle ...

the authors demonstrated that mitochondrial metabolism of pyruvate derived from glucose not only regulates insulin secretion but also directly influences beta-cell growth and plasticity.

CPT1 is responsible for the formation of acylcarnitine from ACADL (acyl-coenzyme A dehydrogenase, long chain), which is responsible for beta-oxidation of fatty acids within the mitochondria.

Oxidative phosphorylation, 7 peroxisomal beta-oxidation of cerotic acid and pristanic acid, 8 and concentrations of very-long-chain fatty acids 9 were measured as described previously.

Mitochondria are essential for generating energy that fuels cells and helps them function. Mitochondrial defects, however, are associated with the development of diseases such as type 2 diabetes.

Besides increased Bcl-2 in the mitochondria of lung macrophages from bleomycin mice, Carter and colleagues also found two proteins — MCU and Cpt1a — showed a similar increase in expression. The MCU ...