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 ...

Bonnefont JP, Bastin J, Behin A, et al. Bezafibrate for an inborn mitochondrial beta-oxidation defect. N Engl J Med . 2009;360(8):838-840. doi: 10.1056/NEJMc0806334 40.

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.

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.

Pyruvate oxidation is critical determinant of pancreatic islet number, beta-cell mass. ScienceDaily . Retrieved June 2, 2025 from www.sciencedaily.com / releases / 2014 / 08 / 140805150851.htm ...

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.

Early role of mitochondria in Alzheimer's Disease may help explain limitations to current beta amyloid hypothesis. ScienceDaily . Retrieved June 3, 2025 from www.sciencedaily.com / releases / 2010 ...

Mitochondrial defects, however, are associated with the development of diseases such as type 2 diabetes. ... Mitochondrial ...