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首页> 外文期刊>Journal of dermatological science >Differential expression of matrix metalloproteinases and proteoglycans in Juvenile Hyaline Fibromatosis.
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Differential expression of matrix metalloproteinases and proteoglycans in Juvenile Hyaline Fibromatosis.

机译：少年透明质酸纤维化病中基质金属蛋白酶和蛋白聚糖的差异表达。

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BACKGROUND: Juvenile Hyaline Fibromatosis (JHF) is a rare autosomal recessive disorder, histologically characterized by the production and deposition of an unidentified hyaline material in the skin and other organs. Extracellular matrix molecules are implicated in the development of skin lesion which is debilitating and recurrent and, so far, no treatment is satisfactory. OBJECTIVE: To investigate the expression of matrix metalloproteinases (MMPs), their tissue inhibitors (TIMPs) and proteoglycans in lesional as compared to site-matched lesion-free skin tissue specimens of a JHF patient, aiming to elucidate the aetiopathological mechanisms involved in the development of JHF skin lesions. METHODS: Gelatinase activity of MMP-2 and MMP-9 was investigated by gelatine zymography. Protein levels of MMP-2, MMP-9, TIMP-1 and TIMP-2 in skin tissue extracts were measured by ELISA. Gene expression of MMPs, TIMPs and proteoglycans was examined by quantitative RT-PCR. RESULTS: JHF lesions exhibited significantly higher activity as well as elevated protein and gene expression of MMP-2 and MMP-9, as compared to lesion-free skin tissue specimens. Decorin was downregulated and aggrecan was upregulated in lesional skin, as compared to normal skin. CONCLUSION: The results presented in this study indicate that MMPs and proteoglycans may be involved in the pathogenesis of JHF and therefore these molecules may offer alternative targets for pharmacological intervention to achieve more radical and effective treatment.
机译：背景：青少年透明质酸纤维瘤病（JHF）是一种罕见的常染色体隐性遗传疾病，其组织学特征是在皮肤和其他器官中产生并沉积了未知的透明质物质。细胞外基质分子与使皮肤衰弱和复发的皮肤病变的发展有关，到目前为止，还没有令人满意的治疗方法。目的：与JHF患者的部位匹配的无病灶皮肤组织标本相比，研究基质金属蛋白酶（MMPs），其组织抑制剂（TIMPs）和蛋白聚糖在病灶中的表达，旨在阐明参与该过程的病因病理机制JHF皮肤病变。方法：采用明胶酶谱法检测MMP-2和MMP-9的明胶酶活性。通过ELISA测定皮肤组织提取物中的MMP-2，MMP-9，TIMP-1和TIMP-2的蛋白质水平。通过定量RT-PCR检查MMP，TIMP和蛋白聚糖的基因表达。结果：与无损伤的皮肤组织标本相比，JHF损伤表现出明显更高的活性以及MMP-2和MMP-9的蛋白质和基因表达升高。与正常皮肤相比，病变皮肤中的Decorin下调，而聚集蛋白聚糖则上调。结论：本研究结果表明MMPs和蛋白聚糖可能参与了JHF的发病机理，因此这些分子可能为药物干预提供替代目标，以实现更彻底和有效的治疗。

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