The pathological changes of the olfactory bulbs in the Niemann-pick type C1 mice

Abstract

Abstract: Objective To examine the impact of Niemann-pick type C1 disease on the olfactory system, we investigated the morphological changes of the olfactory bulbs in NPC1 mutant mouse. Methods The genomic DNA was extracted from mice tails for genotyping by PCR；30-day-old mice including wild type and NPC1 gene mutation were selected, and immunohistochemistry was performed to examine and compare the axonal structure, the myelination process, as well as the expression of SMI31 and MBP in the olfactory bulbs；The cell apoptosis were analyzed by TUNEL assay. Results In NPC1-/- mice, the expression of NPC1 protein is rarely detected, but TUNEL-positive cells are increased. Immunofluorescence using SMI31 antibody indicates accumulation of neurofilament and formation of axonal spheroids in the NPC1-/- olfactory bulbs. Furthermore, the decreased expression of MBP protein is also found in NPC1-/- olfactory system, which is associated with perturbed myelination. Conclusion Our data show pathological changes in the olfactory bulbs of NPC1 mutant mice, which is accompanied by olfactory defects.

Key words: Niemann-pick type C1 disease, olfactory defects, neuron, perturbed myelination, mouse

Xin YAN Liang QIAO Lin Juntang. The pathological changes of the olfactory bulbs in the Niemann-pick type C1 mice[J]. , 2016, 47(1): 2-6.

References

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