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腱生蛋白在正常及囊性人类胎儿肾脏中的分布与发生

Distribution and ontogenesis of tenascin in normal and cystic human fetal kidneys.

作者信息

Daïkha-Dahmane F, Dommergues M, Narcy F, Lacoste M, Gubler M C

机构信息

Inserm U. 423, Hôpital Necker-Enfants Malades, Université René Descartes, Paris, France.

出版信息

Lab Invest. 1995 Oct;73(4):547-57.

PMID:7474927
Abstract

BACKGROUND

Tenascin is a mesenchymal extracellular matrix glycoprotein transiently expressed during development, mainly at the site of epithelial-mesenchymal interactions. It is thought to play a key role in morphogenesis. Little is known about the distribution of tenascin in normal human fetal kidney, and, so far, no data have been reported concerning the distribution of the protein in fetal cystic kidneys.

EXPERIMENTAL DESIGN

Using specific mAb and the immunofluorescence technique, we analyzed the distribution of tenascin in normal human embryonic (n = 3), fetal (n = 15), and mature kidneys (n = 4) and in fetuses affected with autosomal recessive polycystic disease (n = 3), autosomal dominant polycystic disease (n = 3), and cystic dysplasia (n = 3). We compared the distribution of this protein with that of fibronectin and types I, III, V, and VI collagens.

RESULTS

In normal developing kidneys, tenascin is present in the uninduced blastema and in the mesenchyme around differentiating nephrons. It is homogeneously distributed in the inner cortex and in the medulla. During maturation, tenascin expression persists in the medulla but progressively decreases in the cortex. Tenascin is present in the mesangial area from the S-shaped body stage. Both types of polycystic diseases are characterized by a marked and diffuse increase in cortical and medullary expression of tenascin as well as types III, V, and VI collagen. In cystic dysplasia, two types of changes were observed: (a) increased tenascin and interstitial collagen expression in the subcapsular strips of condensed mesenchyme; and (b) heterogeneous medullary tenascin distribution with positive labeling of the condensed mesenchyme surrounding cysts and primitive ducts and negative labeling of the loose interstitial mesenchyme, contrasting with the diffuse accumulation of types III, V, and VI collagen.

CONCLUSIONS

In the human fetal kidney, tenascin is expressed by blastema cells and disappears when converted to epithelium. In polycystic diseases, an early increase in tenascin and interstitial collagen expression suggests that renal mesenchyme per se may contribute to the progressive alteration of the kidney. In cystic dysplasia, phenotypic changes in metanephric blastema indicate inappropriate commitment of blastema cells into interstitial cells, leading to the definitive arrest of nephrogenesis; the heterogeneity in tenascin medullary expression underlines the heterogeneity in the mesenchymal cell population.

摘要

背景

腱生蛋白是一种间充质细胞外基质糖蛋白,在发育过程中短暂表达,主要在上皮 - 间充质相互作用部位表达。它被认为在形态发生中起关键作用。关于腱生蛋白在正常人类胎儿肾脏中的分布知之甚少,并且到目前为止,尚无关于该蛋白在胎儿多囊肾中分布的报道。

实验设计

我们使用特异性单克隆抗体和免疫荧光技术,分析了腱生蛋白在正常人类胚胎肾脏(n = 3)、胎儿肾脏(n = 15)和成熟肾脏(n = 4)以及患有常染色体隐性多囊肾病(n = 3)、常染色体显性多囊肾病(n = 3)和囊性发育异常(n = 3)的胎儿中的分布。我们将该蛋白的分布与纤连蛋白以及Ⅰ型、Ⅴ型和Ⅵ型胶原的分布进行了比较。

结果

在正常发育的肾脏中,腱生蛋白存在于未诱导的胚基以及分化中肾单位周围的间充质中。它在内皮质和髓质中均匀分布。在成熟过程中,腱生蛋白在髓质中持续表达,但在皮质中逐渐减少。从S形期开始,腱生蛋白存在于系膜区。两种类型的多囊肾病的特征均为皮质和髓质中腱生蛋白以及Ⅲ型、Ⅴ型和Ⅵ型胶原的表达显著且弥漫性增加。在囊性发育异常中,观察到两种类型的变化:(a)在浓缩间充质的包膜下条带中腱生蛋白和间质胶原表达增加;(b)髓质中腱生蛋白分布不均,囊肿和原始导管周围的浓缩间充质呈阳性标记,而疏松间质间充质呈阴性标记,这与Ⅲ型、Ⅴ型和Ⅵ型胶原的弥漫性积聚形成对比。

结论

在人类胎儿肾脏中,腱生蛋白由胚基细胞表达,并在转化为上皮细胞时消失。在多囊肾病中,腱生蛋白和间质胶原表达的早期增加表明肾间充质本身可能导致肾脏的渐进性改变。在囊性发育异常中,后肾胚基的表型变化表明胚基细胞不适当分化为间质细胞,导致肾发生最终停滞;髓质中腱生蛋白表达的异质性突显了间充质细胞群体的异质性。

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