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Niemann-Pick Type A Disease: Behavior of Neutral Sphingomyelinase and Vitamin D Receptor
Sphingomyelinase (SMase) is responsible for the breakdown of sphingomyelin (SM) with production of ceramide. The absence of acid sphingomyelinase (aSMase) causes abnormal synapse formation in Niemann-Pick type A (NPA) disease. Because high levels of ceramide in the NPA brain were demonstrated, the i...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6539364/ https://www.ncbi.nlm.nih.gov/pubmed/31086057 http://dx.doi.org/10.3390/ijms20092365 |
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author | Conte, Carmela Arcuri, Cataldo Cataldi, Samuela Mecca, Carmen Codini, Michela Ceccarini, Maria Rachele Patria, Federica Filomena Beccari, Tommaso Albi, Elisabetta |
author_facet | Conte, Carmela Arcuri, Cataldo Cataldi, Samuela Mecca, Carmen Codini, Michela Ceccarini, Maria Rachele Patria, Federica Filomena Beccari, Tommaso Albi, Elisabetta |
author_sort | Conte, Carmela |
collection | PubMed |
description | Sphingomyelinase (SMase) is responsible for the breakdown of sphingomyelin (SM) with production of ceramide. The absence of acid sphingomyelinase (aSMase) causes abnormal synapse formation in Niemann-Pick type A (NPA) disease. Because high levels of ceramide in the NPA brain were demonstrated, the involvement of other SMases were supposed. In the present study we focused the attention on the neurogenic niches in the hippocampal gyrus dentatus (GD), a brain structure essential for forming cohesive memory. We demonstrated for the first time the increase of (Sex determining region Y)-box 2 (SOX2), and the down-regulation of glial fibrillary acidic protein (GFAP) NPA mice GD. Moreover, we found that the expression of Toll like receptors (TLRs), was increased in NPA mice, particularly TLR2, TLR7, TLR8 and TLR9 members. Although no significant change in neutral sphingomyelinase (nSMase) gene expression was detected in the NPA mice hippocampus of, protein levels were enhanced, probably because of the slower protein degradation rate in this area. Many studies demonstrated that vitamin D receptor (VDR) is expressed in the hippocampus GD. Unexpectedly, we showed that NPA mice exhibited VDR gene and protein expression up-regulation. In summary, our study suggests a relation between hippocampal cell differentiation defect, nSMase and VDR increase in NPA mice. |
format | Online Article Text |
id | pubmed-6539364 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-65393642019-06-04 Niemann-Pick Type A Disease: Behavior of Neutral Sphingomyelinase and Vitamin D Receptor Conte, Carmela Arcuri, Cataldo Cataldi, Samuela Mecca, Carmen Codini, Michela Ceccarini, Maria Rachele Patria, Federica Filomena Beccari, Tommaso Albi, Elisabetta Int J Mol Sci Article Sphingomyelinase (SMase) is responsible for the breakdown of sphingomyelin (SM) with production of ceramide. The absence of acid sphingomyelinase (aSMase) causes abnormal synapse formation in Niemann-Pick type A (NPA) disease. Because high levels of ceramide in the NPA brain were demonstrated, the involvement of other SMases were supposed. In the present study we focused the attention on the neurogenic niches in the hippocampal gyrus dentatus (GD), a brain structure essential for forming cohesive memory. We demonstrated for the first time the increase of (Sex determining region Y)-box 2 (SOX2), and the down-regulation of glial fibrillary acidic protein (GFAP) NPA mice GD. Moreover, we found that the expression of Toll like receptors (TLRs), was increased in NPA mice, particularly TLR2, TLR7, TLR8 and TLR9 members. Although no significant change in neutral sphingomyelinase (nSMase) gene expression was detected in the NPA mice hippocampus of, protein levels were enhanced, probably because of the slower protein degradation rate in this area. Many studies demonstrated that vitamin D receptor (VDR) is expressed in the hippocampus GD. Unexpectedly, we showed that NPA mice exhibited VDR gene and protein expression up-regulation. In summary, our study suggests a relation between hippocampal cell differentiation defect, nSMase and VDR increase in NPA mice. MDPI 2019-05-13 /pmc/articles/PMC6539364/ /pubmed/31086057 http://dx.doi.org/10.3390/ijms20092365 Text en © 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Conte, Carmela Arcuri, Cataldo Cataldi, Samuela Mecca, Carmen Codini, Michela Ceccarini, Maria Rachele Patria, Federica Filomena Beccari, Tommaso Albi, Elisabetta Niemann-Pick Type A Disease: Behavior of Neutral Sphingomyelinase and Vitamin D Receptor |
title | Niemann-Pick Type A Disease: Behavior of Neutral Sphingomyelinase and Vitamin D Receptor |
title_full | Niemann-Pick Type A Disease: Behavior of Neutral Sphingomyelinase and Vitamin D Receptor |
title_fullStr | Niemann-Pick Type A Disease: Behavior of Neutral Sphingomyelinase and Vitamin D Receptor |
title_full_unstemmed | Niemann-Pick Type A Disease: Behavior of Neutral Sphingomyelinase and Vitamin D Receptor |
title_short | Niemann-Pick Type A Disease: Behavior of Neutral Sphingomyelinase and Vitamin D Receptor |
title_sort | niemann-pick type a disease: behavior of neutral sphingomyelinase and vitamin d receptor |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6539364/ https://www.ncbi.nlm.nih.gov/pubmed/31086057 http://dx.doi.org/10.3390/ijms20092365 |
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