Telocytes: a potential defender in the spleen of Npc1 mutant mice

Niemann–Pick disease, type C1 (Npc1), is an atypical lysosomal storage disorder caused by autosomal recessive inheritance of mutations in Npc1 gene. In the Npc1 mutant mice (Npc1−/−), the initial manifestation is enlarged spleen, concomitant with free cholesterol accumulation. Telocytes (TCs), a nov...

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Veröffentlicht in:Journal of cellular and molecular medicine 2017-05, Vol.21 (5), p.848-859
Hauptverfasser: Zhang, Bichao, Yang, Ciqing, Qiao, Liang, Li, Qiuling, Wang, Congrui, Yan, Xin, Lin, Juntang
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container_title Journal of cellular and molecular medicine
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creator Zhang, Bichao
Yang, Ciqing
Qiao, Liang
Li, Qiuling
Wang, Congrui
Yan, Xin
Lin, Juntang
description Niemann–Pick disease, type C1 (Npc1), is an atypical lysosomal storage disorder caused by autosomal recessive inheritance of mutations in Npc1 gene. In the Npc1 mutant mice (Npc1−/−), the initial manifestation is enlarged spleen, concomitant with free cholesterol accumulation. Telocytes (TCs), a novel type of interstitial cell, exist in a variety of tissues including spleen, presumably thought to be involved in many biological processes such as nursing stem cells and recruiting inflammatory cells. In this study, we found that the spleen is significantly enlarged in Npc1−/− mice, and the results from transmission electron microscopy examination and immunostaining using three different TCs markers, c‐Kit, CD34 and Vimentin revealed significantly increased splenic TCs in Npc1−/− mice. Furthermore, hematopoietic stem cells and macrophages were also elevated in Npc1−/− spleen. Taken together, our data indicate that splenic TCs might alleviate the progress of splenic malfunction via recruiting hematopoietic stem cells and macrophages.
doi_str_mv 10.1111/jcmm.13024
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In the Npc1 mutant mice (Npc1−/−), the initial manifestation is enlarged spleen, concomitant with free cholesterol accumulation. Telocytes (TCs), a novel type of interstitial cell, exist in a variety of tissues including spleen, presumably thought to be involved in many biological processes such as nursing stem cells and recruiting inflammatory cells. In this study, we found that the spleen is significantly enlarged in Npc1−/− mice, and the results from transmission electron microscopy examination and immunostaining using three different TCs markers, c‐Kit, CD34 and Vimentin revealed significantly increased splenic TCs in Npc1−/− mice. Furthermore, hematopoietic stem cells and macrophages were also elevated in Npc1−/− spleen. 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In the Npc1 mutant mice (Npc1−/−), the initial manifestation is enlarged spleen, concomitant with free cholesterol accumulation. Telocytes (TCs), a novel type of interstitial cell, exist in a variety of tissues including spleen, presumably thought to be involved in many biological processes such as nursing stem cells and recruiting inflammatory cells. In this study, we found that the spleen is significantly enlarged in Npc1−/− mice, and the results from transmission electron microscopy examination and immunostaining using three different TCs markers, c‐Kit, CD34 and Vimentin revealed significantly increased splenic TCs in Npc1−/− mice. Furthermore, hematopoietic stem cells and macrophages were also elevated in Npc1−/− spleen. Taken together, our data indicate that splenic TCs might alleviate the progress of splenic malfunction via recruiting hematopoietic stem cells and macrophages.</abstract><cop>England</cop><pub>John Wiley &amp; Sons, Inc</pub><pmid>27860245</pmid><doi>10.1111/jcmm.13024</doi><tpages>12</tpages><oa>free_for_read</oa></addata></record>
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subjects Animals
Antigens, CD - metabolism
Antigens, CD34 - metabolism
Antigens, Differentiation, Myelomonocytic - metabolism
Autosomal recessive inheritance
CD34 antigen
Cell Count
Cholesterol
Fluorescent Antibody Technique
Hematopoietic stem cells
Hereditary diseases
Heredity
Inflammation
Lipids
Macrophages
Macrophages - metabolism
Male
Medical research
Mice
Mice, Mutant Strains
Mutation
Nanog Homeobox Protein - metabolism
Niemann-Pick disease
Niemann–Pick disease, type C1
Npc1 protein
Nursing
Original
Pluripotent Stem Cells - metabolism
Proteins - metabolism
Proto-Oncogene Proteins c-kit - metabolism
Software
Spleen
Spleen - metabolism
Spleen - pathology
Spleen - ultrastructure
Stem cell transplantation
Stem cells
telocytes
Telocytes - metabolism
Telocytes - pathology
Telocytes - ultrastructure
Transmission electron microscopy
Vimentin
Vimentin - metabolism
title Telocytes: a potential defender in the spleen of Npc1 mutant mice
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