PMID: 3767461Sep 1, 1986Paper

Osteogenesis imperfecta: an x ray fibre diffraction study

Annals of the Rheumatic Diseases
J P Bradshaw, A Miller

Abstract

The use of x ray fibre diffraction to study the molecular architecture of healthy and diseased human tendon is described. The three dimensional structure of human (finger) tendon is derived to high resolution and is shown to be very similar to that reported for rat tail tendon. In particular the presence of the 38 A row line in the diffraction pattern suggests that a high degree of lateral order within the collagen fibrils is a more widespread feature of tendon tissue than was formerly realised. Axially projected electron density maps of the 670 A unit repeat of the collagen fibrils of this tissue, and of tendon tissue from three cases of osteogenesis imperfecta (OI), are calculated and compared. The results are in agreement with recent biochemical studies in suggesting that type I (Sillence) OI is principally a quantitative, rather than a qualitative, defect of type I collagen biosynthesis. The features by which a molecular lesion may be recognised and characterised from diffraction data are discussed.

References

Apr 1, 1979·Journal of Medical Genetics·D O SillenceD M Danks
May 26, 1977·The New England Journal of Medicine·B SykesR Smith
Apr 16, 1971·Nature·A Miller, J S Wray
Aug 9, 1984·The New England Journal of Medicine·D J Prockop, K I Kivirikko
Jun 25, 1983·Journal of Molecular Biology·R D FraserE Suzuki
Aug 1, 1981·Proceedings of the National Academy of Sciences of the United States of America·G S Barsh, P H Byers
Jun 1, 1982·Proceedings of the National Academy of Sciences of the United States of America·G S BarshP H Byers
Jan 1, 1982·Methods in Enzymology·B Brodsky, E F Eikenberry

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Citations

Mar 28, 2009·International Journal of Cancer. Journal International Du Cancer·Veronica J James

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