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Publication : The twitcher mouse: immunocytochemical study of Ia expression in macrophages.

First Author  Higashi Y Year  1992
Journal  J Neuropathol Exp Neurol Volume  51
Issue  1 Pages  47-57
PubMed ID  1740674 Mgi Jnum  J:2124
Mgi Id  MGI:50648 Doi  10.1097/00005072-199201000-00007
Citation  Higashi Y, et al. (1992) The twitcher mouse: immunocytochemical study of Ia expression in macrophages. J Neuropathol Exp Neurol 51(1):47-57
abstractText  The cells expressing immune response associated antigen (Ia) were investigated in the nervous system of the twitcher mouse (an authentic murine model of globoid cell leukodystrophy in humans). With immunocytochemistry using a monoclonal antibody against Mac-1 antigen, many Mac-1 immunoreactive cells (Mac-1 positive cells) were detected in the central as well as the peripheral nervous systems (CNS and PNS). In the CNS, Mac-1 positive cells in the gray matter showed cellular morphology of ramified microglia with delicate cellular processes, while in the white matter Mac-1 positive cells were more plump in shape. Ia expressing cells (Ia positive cells) were also largely confined to the white matter. About 10% of the Mac-1 positive cells were Ia positive. The Ia and Mac-1 positive cells were slender and spindle shaped, and morphologically similar to Ia positive cells in the peripheral nerves while the cells expressing Mac-1 only were more plump in shape. With immunoelectron microscopy, however, both slender Ia positive and plump Ia negative and Mac-1 positive cells revealed electron lucent cytoplasmic vacuoles containing characteristic tubular inclusions of globoid cell leukodystrophy. The results suggest that Ia positive cells are a subset of macrophages in the CNS. Whether Ia expression was induced to endogenous microglia or whether Ia expressing cells were exogenous cells infiltrated in the CNS in response to pathological lesions is yet to be determined.
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