Olfactory deficits in Niemann-Pick type C1 (NPC1) disease.

ABSTRACT:

Background

Niemann-Pick type C disease (NPC) is a rare autosomal recessive lipid storage disease characterized by progressive neurodegeneration. As only a few studies have been conducted on the impact of NPC on sensory systems, we used a mutant mouse model (NPC1(-/-)) to examine the effects of this disorder to morphologically distinct regions of the olfactory system, namely the olfactory epithelium (OE) and olfactory bulb (OB).

Methodology/principal findings

For structural and functional analysis immunohistochemistry, electron microscopy, western blotting, and electrophysiology have been applied. For histochemistry and western blotting, we used antibodies against a series of neuronal and glia marker proteins, as well as macrophage markers. NPC1(-/-) animals present myelin-li

SUBMITTER: Hovakimyan M 

PROVIDER: S-EPMC3877006 | biostudies-literature | 2013

REPOSITORIES: biostudies-literature