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首页> 外文期刊>Cell and Tissue Research >Unexpected matrix diseases and novel therapeutic strategies.
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Unexpected matrix diseases and novel therapeutic strategies.

机译：意外的基质疾病和新颖的治疗策略。

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Within the framework of a broad definition of the extracellular matrix (ECM), this review discusses three genetic disorders in which major pathogenetic features have been traced back to alterations in the levels/activities of matrix components. In each case, disease-associated alterations are found both intra- and extracellularly. The nature of the ECM involvement is surprising, offers an exciting therapeutic opportunity, and deepens our understanding of ECM-cell interactions. The first of these disorders, cherubism, is a case of inflammatory bone loss in the jaws of children for reasons that are surprisingly systemic in nature, considering the local nature of the disease. The primary defect involves an intracellular signaling molecule, but a major pathogenetic component and therapeutic target of the disease is the extracellular cytokine tumor necrosis factor alpha. The second disorder, Knobloch syndrome, is caused by recessive mutations in collagen XVIII. Although this protein has been classified as belonging to a group of structural macromolecules, the consequence of the mutations is impairment of cellular metabolism. The third disorder, infantile hemangioma, is a common tumor of capillary endothelial cells in infancy. The tumor appears within a few days/weeks after birth, grows rapidly over several months, and regresses over several years. The proliferative phase is the result of constitutively high levels of vascular endothelial cell growth factor (VEGF)-dependent signaling through VEGF receptor 2 (VEGFR2), but recent studies have led to the surprising conclusion that abnormalities in a cell-surface receptor complex controlling expression of the VEGF decoy receptor VEGFR1 is the underlying cause.
机译：在细胞外基质（ECM）的广泛定义的框架内，本综述讨论了三种遗传性疾病，其中主要的病原学特征可追溯到基质成分水平/活性的改变。在每种情况下，都在细胞内和细胞外发现与疾病相关的改变。 ECM参与的性质令人惊讶，可提供令人兴奋的治疗机会，并加深了我们对ECM细胞相互作用的理解。考虑到疾病的局部性质，这些疾病中的第一个是红唇病，是儿童颌骨中炎性骨质流失的一种情况，其原因是令人惊讶的系统性疾病。主要缺陷涉及细胞内信号分子，但该疾病的主要致病成分和治疗靶点是细胞外细胞因子肿瘤坏死因子α。第二种疾病，诺氏综合症，是由胶原蛋白XVIII的隐性突变引起的。尽管该蛋白质已被分类为属于结构大分子的一组，但突变的结果是细胞代谢的损害。第三种疾病是婴儿血管瘤，是婴儿期毛细血管内皮细胞的常见肿瘤。肿瘤在出生后几天/几周内出现，在数月内迅速生长，并在数年内消退。增殖期是通过VEGF受体2（VEGFR2）组成的高水平血管内皮细胞生长因子（VEGF）依赖性信号传导的结果，但最近的研究得出了令人惊讶的结论，即细胞表面受体复合物的异常控制表达VEGF诱饵受体VEGFR1的水平升高是根本原因。

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