2-Methylbutyrylcarnitine

2-Methylbutyrylcarnitine is a fatty acid metabolite. 2-Methylbutyrylcarnitine is found mainly in the blood and urine of humans and animals and is produced through the pyruvate carboxylation pathway. 2-Methylbutyrylcarnitine exhibits high level in the plasma of subjects with steatohepatitis (NASH) and can be used as an indicator for the diagnosis of metabolic disorders.