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Substance P immunoreactive cell reductions in cerebral cortex of Niemann-Pick disease type C mouse

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Authors
Kim, Myeung Ju; Kim, Jaewoo; Hutchinson, Brian; Michikawa, Makoto; Cha, Choong Ik; Lee, Bonghee
Issue Date
2005-05-03
Publisher
Elsevier
Citation
Brain Res. 2005 May 10;1043(1-2):218-24.
Keywords
AnimalsCerebral Cortex/cytology/*metabolismFemaleImmunohistochemistryMaleMiceMice, Inbred BALB CMice, Mutant StrainsNeurons/*metabolismNeuropeptide Y/metabolismNiemann-Pick Diseases/*metabolism/pathologySubstance P/*metabolismVasoactive Intestinal Peptide/metabolism
Abstract
Niemann-Pick disease type C (NPC) is characterized by progressive neurodegeneration and arises from mutations in the NPC1 gene. Cholesterol has received most attention in the pathogenesis of NPC, but normalizing lipid levels in humans or mouse does not prevent neurodegeneration. In NPC mouse, neuronal degeneration in the cerebellum is the most commonly detected change, and thus previous studies have tended to focus on the cerebellum, especially Purkinje cells. Although numerous peptides have been found in the mammalian central nervous system, little data on neurotransmitters in NPC are available, and information on neurotransmitter system abnormalities could explain the complex and characteristic deficits of NPC. Thus, we performed an immunohistochemical study on NPC mouse cortices to compare cell numbers exhibiting vasoactive intestinal polypeptide (VIP), neuropeptide Y (NPY), and substance P (SP) immunoreactivity. In terms of VIP and NPY-immunoreactive (ir) cell numbers in the cerebral cortex, SP-ir cells were significantly reduced by about 90% in NPC (-/-) versus NPC (+/+) mouse, and were also mildly decreased in frontal and parietal NPC (+/-) versus NPC (+/+) mouse cortex. This study demonstrates for the first time, reduced number of SP-ir cells in the NPC mouse cortex.
ISSN
0006-8993 (Print)
Language
English
URI
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=15862536

http://hdl.handle.net/10371/22877
DOI
https://doi.org/10.1016/j.brainres.2005.02.052
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College of Medicine/School of Medicine (의과대학/대학원)Anatomy (해부학전공)Journal Papers (저널논문_해부학전공)
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