Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices.
Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices.
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DOI：
10.1083/jcb.124.5.855
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发表时间：
1994-03
期刊：
影响因子：
--
通讯作者：
Ramirez F
中科院分区：
文献类型：
--
作者：
Zhang H;Apfelroth SD;Hu W;Davis EC;Sanguineti C;Bonadio J;Mecham RP;Ramirez F
在以前的克隆的纤维蛋白基因（FBN 1），我们分离出一个部分的cDNA编码的原纤维蛋白样肽和映射相应的基因（FBN 2）到人类5号染色体。(Lee，B.，M.戈弗雷，E. Vitale，H. Hori，M. G. Mattei，M. Sarfarazi，P. Tsipouras，F. Ramirez和D. W.霍利斯特。1991.自然[伦敦]。352：330-334）。然而，这项研究没有解决FBN 2基因产物是否是结构上与Escherin相关的细胞外组分。本报告中介绍的工作澄清了这一重要问题。FBN 2基因产物的整个一级结构的测定表明，该多肽是高度同源的，以甜蛋白。免疫电子显微镜定位两种弹性蛋白相关的细胞外微纤维。最后，免疫组织化学显示，原纤维蛋白共分布在弹性和非弹性结缔组织的发育胚胎，与优先积累的FBN 2基因产物在弹性纤维丰富的矩阵。这些结果支持了原纤维蛋白在细胞外微纤维的形成和维持中可能具有不同但相关的功能的原始假设。因此，我们建议将FBN 1和FBN 2基因产物分类为一个新的细胞外蛋白家族，并分别命名其成员为fetchin-1和fetchin-2。
During the previous cloning of the fibrillin gene (FBN1), we isolated a partial cDNA coding for a fibrillin-like peptide and mapped the corresponding gene (FBN2) to human chromosome 5. (Lee, B., M. Godfrey, E. Vitale, H. Hori, M. G. Mattei, M. Sarfarazi, P. Tsipouras, F. Ramirez, and D. W. Hollister. 1991. Nature [Lond.]. 352:330-334). The study left, however, unresolved whether or not the FBN2 gene product is an extracellular component structurally related to fibrillin. Work presented in this report clarifies this important point. Determination of the entire primary structure of the FBN2 gene product demonstrated that this polypeptide is highly homologous to fibrillin. Immunoelectron microscopy localized both fibrillin proteins to elastin-associated extracellular microfibrils. Finally, immunohistochemistry revealed that the fibrillins co-distribute in elastic and non-elastic connective tissues of the developing embryo, with preferential accumulation of the FBN2 gene product in elastic fiber-rich matrices. These results support the original hypothesis that the fibrillins may have distinct but related functions in the formation and maintenance of extracellular microfibrils. Accordingly, we propose to classify the FBN1 and FBN2 gene products as a new family of extracellular proteins and to name its members fibrillin-1 and fibrillin-2, respectively.