Different fatty acids (FAs) vary significantly in the number of double bonds and the length of the carbon (C) chain. Very long-chain fatty acids (VLCFA) are fatty acids with more than 20 carbon atoms. VLCFA are precursors of lipid mediators and components of cellular lipids such as sphingolipids and glycerophospholipids. Mutations in the genes encoding enzymes involved in VLCFA metabolism can lead to a range of genetic disorders, such as ichthyosis, demyelination, myopathy, intellectual disability, and macular degeneration. By identifying enzymes related to the synthesis and degradation of VLCFA, we can enhance our understanding of the functions of VLCFA.
All plant cells can produce very long-chain fatty acids, which are generated in specific cell types and serve as precursors or components of numerous metabolites. Through the catalysis of a fatty acid elongase complex consisting of four core enzymes located in the endoplasmic reticulum, VLCFA are elongated by adding carbon units to the growing acyl chain. Identification of these enzymes in Arabidopsis has revealed that three of the four enzymes play a production role, producing all VLCFA required for all metabolic pathways, while the fourth enzyme is involved in the condensation reaction, determining specificity and the quantity of synthetic products.
Analysis of Very Long-Chain Fatty Acids
X-linked adrenoleukodystrophy (X-ALD) is a serious progressive degenerative genetic disorder caused by mutations in the ABCD1 gene, leading to a defect in peroxisomes. The lack of peroxisomes results in the accumulation of VLCFA in body tissues, especially in the spinal cord, adrenal glands, and the white matter of the nervous system. This ultimately destroys the myelin that surrounds nerves, causing problems in the nervous system. Although some of the accumulated VLCFA in the body comes from the diet, most are generated by the elongation of medium and long-chain fatty acids within the body. In X-ALD patients, the accumulation of VLCFA is due to a lack of a protein responsible for the degradation of fatty acids. The degradation of VLCFA occurs in peroxisomes within cells, which are present in all cell types in the body except red blood cells. The missing protein is ALDP (X-ALD protein), which is crucial for transporting VLCFA from the cell to the peroxisome.
Biomarkers for patients with peroxisomal disorders (such as X-ALD) are elevated concentrations of VLCFA in plasma and tissues. However, these VLCFA are difficult to quantify simultaneously, and most detection platforms are time-consuming and labor-intensive. Biotage offers reliable, fast, and cost-effective VLCFA analysis services based on highly stable, repeatable, and highly sensitive systems for the separation, characterization, identification, and quantification of VLCFA. Please feel free to inquire!
The VLCFA that Biotage can quantitatively analyze include:
