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Effects of Mutations that Change Primary Structure of Collagen on the Self-Assembly of the Protein into Fibrils

  • Darwin J. Prockop
  • Bruce E. Vogel
  • Reinhard Doelz
  • Jurgen Engel
  • Yoshio Hojima
  • Karl E. Kadler
Conference paper
Part of the Springer Series in Biophysics book series (BIOPHYSICS, volume 3)

Abstract

We have recently observed that a single base mutation in a gene for type I procollagen converts a glycine residue to cysteine and that the substitution for the glycyl residue has a remarkable effect both on the conformation of the molecule and the morphology of the fibrils that are formed as the mutated procollagen molecule is processed to collagen (Vogel et al., 1987; 1988; Kadler et al., 1988b). The observations have largely been made possible through the development of a new system for examining the self-assembly of collagen de novo (Kadler et al., 1987; 1988a).

Keywords

Collagen Fibril Osteogenesis Imperfecta Triple Helix Cysteine Mutation Collagen Triple Helix 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag Berlin Heidelberg 1989

Authors and Affiliations

  • Darwin J. Prockop
    • 1
    • 2
  • Bruce E. Vogel
    • 1
    • 2
  • Reinhard Doelz
    • 3
  • Jurgen Engel
    • 3
  • Yoshio Hojima
    • 1
    • 2
  • Karl E. Kadler
    • 1
    • 2
  1. 1.Department of Biochemistry and Molecular BiologyJefferson Medical CollegePhiladelphiaUSA
  2. 2.Jefferson Institute of Molecular MedicineJefferson Medical CollegePhiladelphiaUSA
  3. 3.The Biocenter of the University of BaselBaselSwitzerland

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