Fibrillin microfibrils

被引：111

作者：

Kielty, CM ^{[1
]}

Sherratt, MJ

Marson, A

Baldock, C

机构：

[1] Univ Manchester, Fac Life Sci, Wellcome Trust Ctr Cell Matrix Res, Manchester M13 9PT, Lancs, England

[2] Univ Manchester, Fac Med & Human Sci, Div Lab & Regenerat Med, Manchester M13 9PT, Lancs, England

来源：

FIBROUS PROTEINS: COILED-COILS, COLLAGEN AND ELASTOMERS | 2005年 / 70卷

关键词：

D O I：

10.1016/S0065-3233(04)70012-1

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Fibrillin microfibrils are widely distributed extracellular matrix assemblies that endow elastic and nonelastic connective tissues with long-range elasticity. They direct tropoelastin deposition during elastic fibrillogenesis and form an outer mantle for mature elastic fibers. Microfibril arrays are also abundant in dynamic tissues that do not express elastin, such as the ciliary zonules of the eye. Mutations in fibrillin-l-the principal structural component of micro-fibrils-cause Marfan syndrome, a heritable disease with severe aortic, ocular, and skeletal defects. Isolated fibrillin-rich microfibrils have a complex 56 nm "beads-on-a-string" appearance; the molecular basis of their assembly and elastic properties, and their role in higher-order elastic fiber formation, remain incompletely understood.

引用

页码：405 / +

页数：37

共 106 条

[1] MOLECULAR-CLONING OF THE MICROFIBRILLAR PROTEIN MFAP3 AND ASSIGNMENT OF THE GENE TO HUMAN-CHROMOSOME 5Q32-Q33.2 [J].