Activation of glial cells in the spinal cord of Niemann-Pick disease type C1 mice

doi:10.16098/j.issn.0529-1356.2017.03.003

Abstract

Abstract:

Objective To explore the impact of Niemann-pick disease type C1 (NPC1) on the developing spinal cord by observing the activation of astrocytes and microglia in different segments of Npc1^-/- spinal cord. Methods Npc1^+/- mice bred to generate Npc1^-/- mice （n=3）and wild type mice (Npc1^+/+ )（n=3）, and the mice genotypes were detected by PCR. Immunofluorescent staining was performed on different levels of spinal cord (cervical, thoracic, lumbar, sacrum) and the activations of astrocytes and microglia were compared between Npc1-/- and Npc1+/+ mice at the postnatal day 35. Double Immunofluorescent staining using GFAP and F4/80 with interleukin-1β(IL-1β) investigated the distribution of IL-1β in the Npc1-/- spinal cord. IL-1β, SMI31 and phos-tau were detected by Western blotting. Results GFAP and F4/80 immunofluorescent staining indicated a significantly increased glial activation (P<0.05) in both dorsal and ventral horn of Npc1^-/- spinal cord, which was associated with enhanced IL-1β expression in the glial cells. Western blotting indicated that an up-regulation of phosphorylated neurofilaments and tau protein resulted in axon accumulation in NPC1^-/- spinal cord. Conclusion Our data show pathological changes of glial cells in the NPC1^-/- spinal cord, which is the possible reason of neuronal defects in the NPC1^-/- mice.

Key words: Niemann-Pick disease type C1, Spinal cord, Astrocyte, Microglia, Western blotting, Mouse

YANG En-hui QIAO Liang LIN Jun-tang YAN Xin. Activation of glial cells in the spinal cord of Niemann-Pick disease type C1 mice[J]. Acta Anatomica Sinica, 2017, 48(3): 260-265.

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