(A) Topologic model depicting NOX1 very early onset
inflammatory bowel disease (VEOIBD) variants (red), selected X-CGD
CYBB (NOX2) variants (green), conserved residues
(blue). (B) Three-dimensional model of NOX1 wild-type
(WT) (grey), NOX1 P330S (green), or NOX1 D360N
(pink) dehydrogenase domains. NADPH, FAD, residue H339, and variant
positions are marked. (C) ROS production by NOX1 WT and
variants. (D) Protein expression of NOX1 and variants, Myc-NOXO1, NOXA1,
and p22phox as loading control. (E) ROS
production in murine Nox1−/− crypts transduced with NOX1 WT or
variants. Phorbol 12-myristate 13-acetate (PMA) stimulation was at 200 seconds.
(F) Localization of Myc-NOX1 WT or D360N (green) in
C. jejuni (red) infected Cos-p22 cells. Scale bar: 10
μm; arrow indicates membrane localization. (G)
Adhesion and invasion of Campylobacter jejuni in cells expressing NOX1
WT, P330S, or D360N. Error bars ± standard deviation n = 3;
*P ≤ .05; **P
≤ .01; ****P ≤ .0001;
comparing NOX1 WT to variants.