title: Telocytes: a potential defender in the spleen of Npc1 mutant mice.
abstract: 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.
authors.affiliation: College of Life Science and Technology, Henan Key Laboratory of Medical Tissue Regeneration, Xinxiang Medical University, Xinxiang, China.
authors.forename: Bichao
authors.initials: B
authors.lastname: Zhang
authors.affiliation: College of Life Science and Technology, Henan Key Laboratory of Medical Tissue Regeneration, Xinxiang Medical University, Xinxiang, China.
authors.forename: Ciqing
authors.initials: C
authors.lastname: Yang
authors.affiliation: College of Life Science and Technology, Henan Key Laboratory of Medical Tissue Regeneration, Xinxiang Medical University, Xinxiang, China.
authors.forename: Liang
authors.initials: L
authors.lastname: Qiao
authors.affiliation: College of Life Science and Technology, Henan Key Laboratory of Medical Tissue Regeneration, Xinxiang Medical University, Xinxiang, China.
authors.forename: Qiuling
authors.initials: Q
authors.lastname: Li
authors.affiliation: College of Life Science and Technology, Henan Key Laboratory of Medical Tissue Regeneration, Xinxiang Medical University, Xinxiang, China.
authors.forename: Congrui
authors.initials: C
authors.lastname: Wang
authors.affiliation: College of Life Science and Technology, Henan Key Laboratory of Medical Tissue Regeneration, Xinxiang Medical University, Xinxiang, China.
authors.forename: Xin
authors.initials: X
authors.lastname: Yan
authors.affiliation: College of Life Science and Technology, Henan Key Laboratory of Medical Tissue Regeneration, Xinxiang Medical University, Xinxiang, China.|College of Biomedical Engineering, Stem Cell and Biotherapy Engineering Research Center of Henan, Xinxiang Medical University, Xinxiang, China.|Institute of Anatomy I, University of Jena School of Medicine, Jena University Hospital, Jena, Germany.
authors.forename: Juntang
authors.initials: J
authors.lastname: Lin
mesh_terms: D000818:Animals; D015703:Antigens, CD / Q000378:metabolism; D018952:Antigens, CD34 / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D002452:Cell Count; D005455:Fluorescent Antibody Technique; D047908:Intracellular Signaling Peptides and Proteins; D008264:Macrophages / Q000378:metabolism; D008297:Male; D008817:Mice, Mutant Strains; D000071317:Nanog Homeobox Protein / Q000378:metabolism; D000077296:Niemann-Pick C1 Protein; D039904:Pluripotent Stem Cells / Q000378:metabolism; D011506:Proteins / Q000378:metabolism*; D019009:Proto-Oncogene Proteins c-kit / Q000378:metabolism; D013154:Spleen / Q000378:metabolism* / Q000473:pathology / Q000648:ultrastructure; D000067170:Telocytes / Q000378:metabolism* / Q000473:pathology / Q000648:ultrastructure; D014746:Vimentin / Q000378:metabolism; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D018952:Antigens, CD34; D015214:Antigens, Differentiation, Myelomonocytic; D047908:Intracellular Signaling Peptides and Proteins; D000071317:Nanog Homeobox Protein; D000077296:Niemann-Pick C1 Protein; D011506:Proteins; D019009:Proto-Oncogene Proteins c-kit; D014746:Vimentin; C067980:CD68 antigen, human; D000097382:CD68 Molecule; C475540:Nanog protein, mouse; C106831:Npc1 protein, mouse
keywords: Niemann-Pick disease, type C1; hematopoietic stem cells; macrophages; spleen; telocytes