系统性硬化症（硬皮病）患者一级亲属的真皮成纤维细胞培养物中的原纤维蛋白1异常。 - Suppr | 超能文献

Fibrillin 1 abnormalities in dermal fibroblast cultures from first-degree relatives of patients with systemic sclerosis (scleroderma).

作者信息

Wallis Debra D, Tan Filemon K, Kessler Rebecca, Kimball Misty D, Cretoiu Jill S, Arnett Frank C, Milewicz Dianna M

机构信息

University of Texas-Houston Medical School.

出版信息

Arthritis Rheum. 2004 Jan;50(1):329-32. doi: 10.1002/art.11437.

DOI:10.1002/art.11437

Abstract

摘要

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Fibrillin 1 abnormalities in dermal fibroblast cultures from first-degree relatives of patients with systemic sclerosis (scleroderma).系统性硬化症（硬皮病）患者一级亲属的真皮成纤维细胞培养物中的原纤维蛋白1异常。

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Mutations in fibrillin-1 cause congenital scleroderma: stiff skin syndrome.原纤维蛋白-1 突变导致先天性硬皮病：硬皮病综合征。

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Fibrillin in Marfan syndrome and tight skin mice provides new insights into transforming growth factor-beta regulation and systemic sclerosis.马凡综合征和紧皮小鼠中的原纤蛋白为转化生长因子-β调节和系统性硬化症提供了新见解。

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Mutant fibrillin 1 from tight skin mice increases extracellular matrix incorporation of microfibril-associated glycoprotein 2 and type I collagen.来自紧皮小鼠的突变原纤蛋白1增加了微原纤维相关糖蛋白2和I型胶原在细胞外基质中的掺入。

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Assembly of epithelial cell fibrillins.上皮细胞原纤蛋白的组装。

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Keratinocytes express fibrillin and assemble microfibrils: implications for dermal matrix organization.角质形成细胞表达原纤维蛋白并组装微原纤维：对真皮基质组织的影响。

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Arthritis Res Ther. 2013 Aug 20;15(4):R90. doi: 10.1186/ar4270.

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Elevated expression of isopeptide bond cross-links contributes to fibrosis in scleroderma and the healing wounds of tight skin mice.异肽键交联的表达升高会导致硬皮病和紧皮小鼠愈合伤口中的纤维化。

Wound Repair Regen. 2008 Sep-Oct;16(5):699-705. doi: 10.1111/j.1524-475X.2008.00420.x.