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VLCDA clinical mutation can lead to disease state. VLCAD is used to break down very long-chain fatty acids, and they are found in food and body’s fat tissue [[1]]. Fatty acids play a crucial role which provides energy for heart and muscle [[2]]. Thus, VLCAD deficiency can cause severe neonatal cardiomyopathy and liver failure which occur mostly in adolescence or adulthood [[2]]. In addition, if the body does not have sufficient amount of VLCAD, it would affect the metabolism of the body [[1]]. There are several mutation sites that have been found. Mutation R429W is severe in childhood phenotype [[2]]. Arg-429 on the helix K makes salt-bridge with Glu-384 on helix I [[2]]. The enzyme is destabilized, and the salt bridge is broken due to the replacing charged residue with the bulky neutral residue [[2]]. Another mutation site is R416H which is found on the helix J [[2]]. Both sites, R429 and R416H, are located close the catalytic glutamate [[2]]. Site R416H has Arg-416 interacts with Asp 391 by making salt bridge and interacts with Gln-395 though hydrogen bond [[2]]. Mutation in R416H causes problem to the position of helix J [[2]]. Furthermore, forming salt bridge with the opposing monomer can affect the dimer interaction [[2]].  

Deficient VLCAD affects 1 person in 40,000 to 120,000 people, thus, it is a very rare diseases [[1]].