The long-chain fatty acid elongases (ELOVL) catalyse the first rate-limiting step in the two carbon elongation of the acyl chains of fatty acids (FAs) greater than 12 carbons in length. Defects in these ELOVL elongases cause severe genetic diseases, such as Stargardt disease-3 and several ataxias, and knockout studies suggest roles in insulin resistance and hepatic steatosis. This TEP provides the first structural information for this family of enzymes which, coupled with mutagenesis and biophysical studies, demonstrates how substrates and products bind within the active site.

Future versions of this TEP will contain experimental data on the ELOVL7 TEP.