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NOTCH 通路在婴幼儿血管瘤中的激活。

NOTCH pathway activation in infantile hemangiomas.

机构信息

Department of Otolaryngology and Head and Neck Surgery, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, Ark.

Department of Otolaryngology and Head and Neck Surgery, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, Ark.

出版信息

J Vasc Surg Venous Lymphat Disord. 2021 Mar;9(2):489-496. doi: 10.1016/j.jvsv.2020.07.010. Epub 2020 Aug 5.

Abstract

OBJECTIVE

The objective of this study was to characterize the role of NOTCH signaling cascade activation in the angiogenic drive of infantile hemangioma (IH) tissue.

METHODS

Both IH tissue and normal skin were collected from 55 surgical patients. Of these, 14 were proliferating hemangiomas, 14 were stationary, 14 were involuted, and 13 had been treated with propranolol. Protein and RNA were extracted from all tissues and subjected to Western blotting and reverse transcription-polymerase chain reaction, respectively, for components of the NOTCH signaling pathway.

RESULTS

Compared with normal skin from the same patients, proliferating IHs contained higher levels of messenger RNA and protein for the majority of NOTCH receptors and ligands as well as the downstream coactivator MAML1. Expression of NOTCH receptor ligand messenger RNA and protein was significantly lower in involuting and propranolol-treated IHs.

CONCLUSIONS

Perturbations in NOTCH signaling follow the natural course and treatment outcome of IHs, suggesting a critical role in their pathogenesis.

摘要

目的

本研究旨在探讨 NOTCH 信号级联激活在婴幼儿血管瘤(IH)组织血管生成中的作用。

方法

收集 55 例手术患者的 IH 组织和正常皮肤。其中增殖期血管瘤 14 例,稳定期 14 例,消退期 14 例,13 例已用普萘洛尔治疗。所有组织均提取蛋白质和 RNA,分别进行 Western blot 和逆转录-聚合酶链反应,检测 NOTCH 信号通路的组成部分。

结果

与来自同一患者的正常皮肤相比,增殖期 IH 组织中大多数 NOTCH 受体和配体以及下游共激活因子 MAML1 的信使 RNA 和蛋白水平更高。在消退期和普萘洛尔治疗的 IH 中,NOTCH 受体配体信使 RNA 和蛋白的表达显著降低。

结论

NOTCH 信号的紊乱遵循 IH 的自然病程和治疗结果,提示其在发病机制中起关键作用。

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