ASSEMBLY OF CARTILAGE COLLAGEN FIBRILS IS DISRUPTED BY OVEREXPRESSION OF NORMAL TYPE-II COLLAGEN IN TRANSGENIC MICE

被引：51

作者：

GAROFALO, S ^{[1
]}

METSARANTA, M ^{[1
]}

ELLARD, J ^{[1
]}

SMITH, C ^{[1
]}

HORTON, W ^{[1
]}

VUORIO, E ^{[1
]}

DECROMBRUGGHE, B ^{[1
]}

机构：

[1] UNIV TEXAS,HLTH SCI CTR,DEPT PEDIAT,HOUSTON,TX 77030

来源：

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA | 1993年 / 90卷 / 09期

关键词：

D O I：

10.1073/pnas.90.9.3825

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

Cartilage collagen fibrils, which are characterized by their thin, uniform diameters, are formed of a multicomponent system of three collagen types (II, IX, and XI) and interacting proteoglycans. We have used a genetic approach to test whether the proper assembly of this multiprotein structure was altered by overexpression of one of its normal components. Here we show that in transgenic mice in which the normal mouse alpha1(II) collagen is overexpressed, thick abnormal collagen fibrils are generated. Mice that showed the highest expression of the transgene also displayed a larger proportion of abnormal fibrils and died at birth. We propose that an imbalance among the constituents of the cartilage collagen fibrils disrupts the mechanism that controls their assembly. The results show the applicability of the transgenic mice system to studies of complex multicomponent protein assemblies in intact animals.

引用

页码：3825 / 3829

页数：5

共 25 条

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[Anonymous], 1986, MANIPULATING MOUSE E

[2]

EIKENBERRY EF, 1992, ARTICULAR CARTILAGE AND OSTEOARTHRITIS, P133

[3]

FJOLSTAD M, 1974, J PATHOL, V112, P183, DOI 10.1002/path.1711120309

[4] REDUCED AMOUNTS OF CARTILAGE COLLAGEN FIBRILS AND GROWTH PLATE ANOMALIES IN TRANSGENIC MICE HARBORING A GLYCINE-TO-CYSTEINE MUTATION IN THE MOUSE TYPE-II PROCOLLAGEN ALPHA-1-CHAIN GENE [J].