DOI: 10.1073/pnas.94.5.1852. Type III collagen is crucial for collagen I fibrillogenesis and for normal cardiovascular development

Type III collagen is crucial for collagen I fibrillogenesis and for normal cardiovascular development

10.1073/pnas.94.5.1852

Crossref journal-article

Proceedings of the National Academy of Sciences

Proceedings of the National Academy of Sciences (341)

Abstract

Type III collagen is a fibrillar forming collagen comprising three α1(III) chains and is expressed in early embryos and throughout embryogenesis. In the adult, type III collagen is a major component of the extracellular matrix in a variety of internal organs and skin. Mutations in the COL3A1 gene have been implicated as a cause of type IV Ehlers–Danlos syndrome, a disease leading to aortic rupture in early adult life. To directly study the role of Col3a1 in development and disease, we have inactivated the Col3a1 gene in embryonic stem cells by homologous recombination. The mutated allele was transmitted through the mouse germ line and homozygous mutant animals were derived from heterozygous intercrosses. About 10% of the homozygous mutant animals survived to adulthood but have a much shorter life span compared with wild-type mice. The major cause of death of mutant mice was rupture of the major blood vessels, similar to patients with type IV Ehlers–Danlos syndrome. Ultrastructural analysis of tissues from mutant mice revealed that type III collagen is essential for normal collagen I fibrillogenesis in the cardiovascular system and other organs.

Bibliography

Liu, X., Wu, H., Byrne, M., Krane, S., & Jaenisch, R. (1997). Type III collagen is crucial for collagen I fibrillogenesis and for normal cardiovascularâdevelopment. Proceedings of the National Academy of Sciences, 94(5), 1852â1856.

Authors 5

Xin Liu (first)
Hong Wu (additional)
Michael Byrne (additional)
Stephen Krane (additional)
Rudolf Jaenisch (additional)

References 27 Referenced 494

10.1056/NEJM198408093110606
10.1172/JCI114863
10.1111/1523-1747.ep12320704
10.1007/BF01931087
10.1016/0003-4975(95)00706-Q
10.1146/annurev.bi.59.070190.004201
K Piez Extracellular Matrix Biochemistry, eds K Piez, A Reddi (Elservier, New York), pp. 1–40 (1984). / Extracellular Matrix Biochemistry by Piez K (1984)
10.1016/B978-0-12-481280-2.50005-2
10.1016/S0021-9258(19)40644-3
10.1146/annurev.bi.49.070180.004521
10.1073/pnas.80.11.3354
10.1177/36.11.3049791
10.1016/0022-1759(77)90168-5
10.1073/pnas.78.12.7360
10.1016/S0021-9258(19)46970-6
10.1016/S0021-9258(17)30289-2
10.1042/bj2680339
10.1007/978-1-4613-2831-5_2
10.1083/jcb.105.5.2393
J Uitto, E A Bauer Diseases Associated with Collagen Abnormalities (Churchill Livingstone, New York, 1982). / Diseases Associated with Collagen Abnormalities by Uitto J (1982)
10.1073/pnas.91.7.2819
10.1016/0092-8674(92)90611-F
J D Bancroft, H C Cook Manual of Histological Techniques (Churchill Livingston, New York), pp. 49–51 (1984). / Manual of Histological Techniques by Bancroft J D (1984)
10.1073/pnas.91.11.5070
10.1038/ng0195-31
10.1083/jcb.130.1.227
10.1101/gad.9.22.2821

Dates

Type	When
Created	23 years, 1 month ago (July 26, 2002, 10:31 a.m.)
Deposited	3 years, 4 months ago (April 13, 2022, 2:16 p.m.)
Indexed	1 day, 9 hours ago (Aug. 31, 2025, 6:15 a.m.)
Issued	28 years, 5 months ago (March 4, 1997)
Published	28 years, 5 months ago (March 4, 1997)
Published Online	28 years, 5 months ago (March 4, 1997)
Published Print	28 years, 5 months ago (March 4, 1997)

Funders 0

None

BibTeX

@article{Liu_1997, title={Type III collagen is crucial for collagen I fibrillogenesis and for normal cardiovascular development}, volume={94}, ISSN={1091-6490}, url={http://dx.doi.org/10.1073/pnas.94.5.1852}, DOI={10.1073/pnas.94.5.1852}, number={5}, journal={Proceedings of the National Academy of Sciences}, publisher={Proceedings of the National Academy of Sciences}, author={Liu, Xin and Wu, Hong and Byrne, Michael and Krane, Stephen and Jaenisch, Rudolf}, year={1997}, month=mar, pages={1852–1856} }

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