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. Author manuscript; available in PMC: 2016 Nov 1.
Published in final edited form as: Mol Genet Metab. 2015 Sep 2;116(3):139–145. doi: 10.1016/j.ymgme.2015.08.011

Fig. 3. Structural analysis of VLCAD variants.

Fig. 3

Enriched VUSs, described in Table 1, were plotted on the crystal structure of the VLCAD homodimer (PDB# 2UXW) where the two VLCAD moieties are indicated by yellow and blue ribbon structures. Red spheres indicate the location of VUSs. Stick diagrams indicate the location of the substrate (Palmitoyl-CoA) and the cofactor, flavin adenine dinucleotide (FAD). The location of the catalytic residue, Glu462, and the p.V283A pathogenic variant are shown by green spheres.