Pathologic changes of glial cells in murine model of Niemann-Pick disease type C: immunohistochemical, lectin-histochemical and ultrastructural observations.

Background: In recent years, morbid states of glial cells have been reported in several neurodegenerative diseases. We studied neuropathologically the glial cells in a murine model of Niemann-Pick disease type C (NPC) to clarify involvement of glias, the most important supportive cells in the central nervous system, by the disease.

Methods: The brains of sphingomyelinosis mice (spm/spm), aged from 5 to 13 weeks, and 15 of their age-matched normal siblings were studied histopathologically, immunohistochemically and electron micro-scopically.