Figure 2.
Figure 2. GOI interfaces in pathogenic SOD1 give rise to cross-
fibrils
in two different crystal systems. (a) Orthogonal views of the
linear, amyloid-like filaments represented by three dimers shown
from top to bottom in green, gold and blue, respectively. Both
the S134N and apo H46R linear filaments are represented by the
single filament shown in panels i -iv. The GOI interface is red
in the filament in i and boxed in ii -iv. In iv, which is
rotated 90° relative to ii and iii, -strands
1, 2, 3 and 6, comprising one-half of each SOD1 -barrel,
are shown in red. The 'cross- '
structure observed in amyloid fibrils is shown schematically in
v. (b) Stereo view of the GOI interface in the S134N filament.
Residues 125 -131 of the electrostatic loop from one S134N dimer
(orange) interact with a depression in the -barrel
of a neighboring S134N dimer (green) in the crystal lattice.
Water molecules are represented as black spheres. The 1.3 Å [A]^55-weighted
electron density, with coefficients 2mF[o] - DF[c], is contoured
at 1.0 .
(c) Small hydrophobic core formed at the GOI interface in the
linear, pathogenic SOD1 filaments (see text). The image is an
enlargement of the region boxed in image iii of panel a.
fibrils
in two different crystal systems. (a) Orthogonal views of the
linear, amyloid-like filaments represented by three dimers shown
from top to bottom in green, gold and blue, respectively. Both
the S134N and apo H46R linear filaments are represented by the
single filament shown in panels i -iv. The GOI interface is red
in the filament in i and boxed in ii -iv. In iv, which is
rotated 90° relative to ii and iii, 
-strands
1, 2, 3 and 6, comprising one-half of each SOD1 
[A]^55-weighted
electron density, with coefficients 2mF[o] - DF[c], is contoured
at 1.0