Xrcc4 mutation drives enhanced susceptibility to senescence.
(a) Three-dimensional structure of the Lig4 complex, determined
using the human structure as scaffolding. Modified residues are highlighted in
red, and the arrow and dotted lines indicate regions of major instability. The
box highlights the regions shown in detail in b and c.
(b,c) Molecular interactions calculated along the represented
trajectory for the B10 (b) and NOD (c) alleles of
Xrcc4. (d) Per-residue root-mean-square
fluctuation (RMSF) in Xrcc4 between 70–100 ns of simulation. Regions
with a different fluctuation profile are highlighted by the dashed lines and one
arrow, corresponding to the regions indicated in a. The results are
representative of four simulations. (e,f) Representative
immunoblotting of mouse embryonic fibroblasts (MEFs) for Xrcc4, Xlf and Lig4
(e), with quantification (n = 5
technical replicates/group) (f). (g) Proportion of
MEFs that remained positive for H2A.X (Ser139) phosphorylation (pH2A.X;
indicative of DSBs) after etoposide exposure (n = 6
technical replicates/group). (h) Wild-type CHO cells and
Xrcc4-deficient (KO) CHO cells reconstituted with the B10
or NOD Xrcc4 allele were exposed to etoposide, and unrepaired
DNA damage was quantified (n = 6–8 technical
replicates/group). (i,j) Immunofluorescence with a polyclonal
antibody to insulin, antibody to phosphorylated H2A.X and DAPI on pancreata from
the B10k.Rag1−/− and
NODk.Rag1−/−
backgrounds. Quantification is shown for islet raw fluorescence in the channel
for phosphorylated H2A.X (n = 19, 17, 20 and 20
sections) (i), with images of representative sections
(j). Scale bar, 100 μm. (k) Diabetes
incidence of (B10k × DBA/2)F1 and (B10k ×
DBA/2)F1.insHEL mice (n = 6 and 11 mice).
(l,m) Immunofluorescence with a polyclonal antibody to insulin,
antibody to phosphorylated H2A.X and DAPI on pancreata from the B10k
and (B10k × DBA/2)F1 backgrounds. Quantification is shown of
islet raw fluorescence in the channel for phosphorylated H2A.X
(n = 19, 17, 16 and 20 sections) (l), with images
of representative sections (m). Scale bar, 100 μm. Data are shown as
means ± s.e.m. *P < 0.05,
**P < 0.01,
***P < 0.0001; NS, not
significant.