原纤维蛋白1抗体
产品名称: 原纤维蛋白1抗体
英文名称: Fibrillin 1
产品编号: bs-1157R
产品价格: null
产品产地: 北京
品牌商标: BIOSS
更新时间: 2025-12-02T14:02:48
使用范围: ELISA=1:500-1000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,
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This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome. [provided by RefSeq, Jul 2008]
