Telocytes: a potential defender in the spleen of Npc1 mutant mice.
Telocytes: a potential defender in the spleen of Npc1 mutant mice.
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特洛细胞：Npc1 突变小鼠脾脏中的潜在防御者。
DOI：
10.1111/jcmm.13024
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发表时间：
2017-05
影响因子：
5.3
通讯作者：
Lin J
中科院分区：
文献类型：
--
作者：
Zhang B;Yang C;Qiao L;Li Q;Wang C;Yan X;Lin J
Niemann-Pick病，C1型(NPC1)，是一种由NPC1基因突变常染色体隐性遗传引起的非典型溶酶体储存障碍。在npc1突变小鼠(npc1−/−)中，最初的表现是脾增大，伴随着游离胆固醇的积累。端粒细胞(TCs)是一种新型的间质细胞，存在于包括脾在内的多种组织中，被认为参与了许多生物学过程，如哺育干细胞和募集炎症细胞。在本研究中，我们发现NPC1−/−小鼠的脾显著增大，并且通过对c-Kit、CD34和Vimentin三种不同的TCS标记物的透射电子显微镜检查和免疫组织化学染色，发现NPC1−/−小鼠的脾Tcs显著增加。此外，NPC1−/−小鼠脾中的造血干细胞和巨噬细胞也明显增多。综上所述，我们的数据表明，脾TCS可能通过招募造血干细胞和巨噬细胞来缓解脾功能障碍的进展。
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.