2-methylbutyroylcarnitine

Name: 2-methylbutyroylcarnitine

Description: 2-Methylbutyroylcarnitine is a member of the class of compounds known as acylcarnitines. Acylcarnitines are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine ... through an ester bond. Thus, 2-Methylbutyroylcarnitine is considered to be a fatty ester lipid molecule. It is a C5-acylcarnitine having 2-methylbutyryl as the acyl substituent. 2-Methylbutyroylcarnitine is practically insoluble (in water) and a weakly acidic compound (based on its pKa). 2-Methylbutyroylcarnitine can be found in urine. Within the cell, 2-Methylbutyroylcarnitine is primarily located in the membrane (predicted from logP). It can also be found in the extracellular space. Usually not detected in normal individuals, the elevation of 2-methylbutyrylcarnitine suggests a deficiency of a dehydrogenase specific for isobutyryl-CoA, important in the differential diagnosis of branched chain organic acidurias by analysis of urinary organic acids and acylcarnitines in plasma or dried blood spots (The Metabolic and Molecular Bases of Inherited Disease, Chapter 93)