Figure 2. Skeletal and Neurological Abnormalities in Csb^m/m/Xpa^−/− Mice.

(A) Radiographs of wt and Csb^m/m/Xpa^−/− mice (age as indicated) and photograph of a 15-d-old Csb^m/m/Xpa^−/− mouse (C57BL/6J).

(B) 2-D images of micro-CT scans of tibiae taken from Csb^m/m/Xpa^−/− animals and wt littermate controls (age indicated in the figure). Horizontal sections are shown of the upper and lower part of the tibiae. e, epiphysis; g, growth plate; and d, diaphysis. I indicates the section through the smaller part of the diaphysis, II indicates section through the broader part of the diaphysis.

(C) Growth of tibiae, taken from Csb^m/m/Xpa^−/− animals (light squares) and wt littermate controls (dark squares).

(D) Representative footprint patterns of 19-d-old wt and Csb^m/m/Xpa^−/− mice. Arrows indicate the trajectory of each mouse. Stride length and front base width measurements on 15-d-old wt, Xpa^−/−, Csb^m/m, and Csb^m/m/Xpa^−/− mice. The significantly (asterisk; p < 0.001) greater base width in the double mutant mouse indicates ataxia.

(E) Representative pictures of a TUNEL staining in the retina of wt and Csb^m/m/Xpa^−/− mice and quantification of the number of TUNEL positive cells. Arrows indicate TUNEL positive cells in the ONL and INL. Note the significantly higher number of TUNEL-positive cells in both the ONL and the INL in the retina of Csb^m/m/Xpa^−/− compared to wt mice (asterisk; p < 0.05).