view article
Figure 5
Structural bases of Alport's and Goodpasture's syndromes. (a) Goodpasture's epitopes EA (left) and EB (right) as seen in the superimposed structures of subunits of α3NC1homo (cyan or blue) and α5NC1homo (orange or green). The same colour code is used for the amino-acid side chains (except for invariant residues, which are shown in black). (b) Structure-assisted sequence alignment of the α1–5NC1 chains. Arrows indicate β-strands and cylinders indicate α-helices. β-Strands of the I/I′ and II/II′ sheets are coloured light pink and light blue, respectively. Blue rectangles enclose flexible regions and Cl motifs (labelled), horizontal magenta lines mark the EA and EB epitopes (also labelled) and coloured shadowing indicates the residues reported to host missense mutations in Alport's syndrome. (c) Alport's syndrome missense mutations (listed in Supplementary Table S5) are mapped onto the structure of an α5NC1 subunit. Yellow spheres represent mutations in this chain, while superimposed mutations of α3NC1 and α4NC1 are coloured orange and green. β-Sheets I/I′ and II/II′ are coloured light pink and light blue, respectively, EA and EB are shown in magenta and flexible SM regions and Cl motifs are presented in deep blue.

IUCrJ
Volume 5| Part 6| November 2018| Pages 765-779
ISSN: 2052-2525
https://doi.org/10.1107/S2052252518012459