Phenotypic features of rib cartilage cells in funnel-shaped chest deformity

E. L. Strokova, N. Pakhomova, A. I. Shevchenko, A. A. Korytkin, V. V. Kozhevnikov, А. M. Zaidman
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Abstract

Funnel-shaped deformity of the chest is a serious disease with an unknown etiology and pathogenesis. Pathological changes occurring in the cartilaginous tissue of the ribs are one of the leading mechanisms for the development of chest curvature. Analysis of the phenotypic features of cells isolated from the site of localization of the pathological process will create a more complete picture of the deformation development.Aim of the study was to identify the phenotype of rib cartilage cells in funnel-shaped deformation of the thorax.Material and methods. Rib cartilage cells of children with funnel-shaped chest deformity were cultured up to the 2–3rd passage, then were identified by histochemical and immunohistochemical methods.Results and discussion. The study revealed cells of two different phenotypes. The first type is oval and polygonal chondroblasts expressing a marker of chondrogenic differentiation. The second type is the cells of the neural phenotype with the expression of early neural markers Musashi1 and PAX6 and the neurospecific protein III β-tubulin, as well as the substance Nissl.Conclusions. The analysis of the phenotypic features of rib cartilage cells with funnel-shaped curvature of the chest allowed us to expand the idea of a possible etiological factor in the deformation development.
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漏斗状胸部畸形中肋软骨细胞的表型特征
胸部漏斗状畸形是一种病因和发病机制不明的严重疾病。肋骨软骨组织发生的病理变化是导致胸部弯曲的主要机制之一。分析从病理过程定位部位分离出的细胞的表型特征将能更全面地了解变形的发展过程。研究目的是确定胸部漏斗状变形中肋软骨细胞的表型。将漏斗状胸部畸形患儿的肋软骨细胞培养至第 2-3 期,然后通过组织化学和免疫组化方法进行鉴定。研究发现了两种不同表型的细胞。第一种是表达软骨分化标记的椭圆形和多角形软骨母细胞。第二种是神经表型细胞,表达早期神经标志物 Musashi1 和 PAX6、神经特异性蛋白 III β-tubulin,以及 Nissl 物质。通过对胸部漏斗状弯曲的肋软骨细胞表型特征的分析,我们拓展了畸形发生的可能病因的思路。
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