The twitcher mouse: positive immunohistochemical staining of globoid cells with monoclonal antibody against Mac-1 antigen.
The expression of Mac-1 antigen (macrophage surface antigen) was studied by the immunoperoxidase method in the nervous system of the twitcher mouse (an authentic murine model of globoid cell leukodystrophy) from day 10 to day 39, to elucidate the origin of the globoid cells. Mac-1 positive cells were seen in peripheral nervous system (PNS) at all ages examined. The positive cells in central nervous system (CNS) showed staining characteristics similar to those in PNS, but in the CNS positive cells they appeared first at day 15 and increased significantly in older mice. These cells were always predominant in the white matter. There were few positive cells in the neural parenchyma of control mice before day 10 but none thereafter. The occurrence and the preferential distribution indicate that it is quite likely that Mac-1 positive cells in the twitcher mouse represent globoid cells. Twitcher mice which were previously given colloidal carbon intravenously, had some Mac-1 positive cells which contained carbon particles both in the CNS and PNS. These facts indicate that globoid cells are the cells of monocytic lineage and at least some of them are derived from blood monocytes.
D000818:Animals; D000954:Antigens, Surface; D004195:Disease Models, Animal; D006801:Humans; D007124:Immunoenzyme Techniques; D007965:Leukodystrophy, Globoid Cell / Q000276:immunology* / Q000473:pathology; D016177:Macrophage-1 Antigen; D051379:Mice; D008810:Mice, Inbred C57BL; D008818:Mice, Neurologic Mutants; D009000:Monocytes / Q000276:immunology*; D009420:Nervous System / Q000473:pathology; D010586:Phagocytes / Q000276:immunology*
D000954:Antigens, Surface; D016177:Macrophage-1 Antigen