Fibrillin controls TGF-β activation

被引：118

作者：

Kaartinen, V ^{[1
]}

Warburton, D

机构：

[1] Univ So Calif, Childrens Hosp Los Angeles, Dev Biol Program, Inst Res,Keck Sch Med, Los Angeles, CA 90027 USA

[2] Univ So Calif, Sch Dent, Los Angeles, CA 90027 USA

来源：

NATURE GENETICS | 2003年 / 33卷 / 03期

关键词：

D O I：

10.1038/ng0303-331

中图分类号：

Q3 [遗传学];

学科分类号：

071007 ; 090102 ;

摘要：

Secreted transforming growth factor-βs (TGF-βs) are rendered biologically inactive by binding proteins that also target and concentrate them to the extracellular matrix. Specific, but still poorly understood, activation is required for disassembly of the extracellular matrix–bound protein complex to liberate the mature growth factor and to obtain a correct biological effect. A new study shows that fibrillin-1, the protein defective in Marfan syndrome, has a biologically important role in controlling TGF-β activation in the lung.

引用

页码：331 / 332

页数：2

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