Modeling of 17 pathogenic mutations in the spectrin self-association domain.

(A) Mutations involving αΙ-spectrin. (B) Mutations involving βΙ-spectrin. Note the predicted disruption, sometimes severe, that accompanies the substitution of even a single native residue. Longitudinal and end-on views are shown for each mutation. The mutated residue is depicted in each case using a solid-filled representation. The coordinates for each of these modeled structures are available as .PDB files (downloadable as .RTF files) on the Blood website as supplemental material; see the Supplemental Data Sets link at the top of the online article.