The aim of the present work was to further characterize immunological
defects in osteopetrosis. The op/op mutant mouse is of particular inte
rest since a marrow cavity develops spontaneously in older animals. Th
e interleukin production (IL-1, IL-2, IL-3, IL-4, IL-6), the presence
of macrophage differentiation antigens and the evolution of the bone l
esions were studied in osteopetrotic and normal mice. Low levels of IL
-l, IL-3 and IL-4 were observed at the age of 6 weeks in the op/op mic
e. However, at 22 weeks of age, the level of IL-I and IL-4 returned to
normal value in these op/op mice whereas the level of IL-3 remained p
artially decreased at the same age. Furthermore, macrophage expression
of MAC-2 antigen, reduced at 12 weeks of age was found to be normal 1
0 weeks later. These immunological defects and their recovery seems to
be concomitant with the healing of the bone lesions.