Collagen production by skin fibroblast cell strains from normal subjects and age-matched patients with the mendelian disorder--Osteogenesis Imperfecta (OI)--was studied in culture. / Number of generations in culture, phase of growth, labelling times, and site of biopsy did not influence collagen production by normal skin fibroblasts. / OI cell strains from patients with dominantly inherited OI have abnormal morphology and growth in culture. The ratio of Type I to protype III collagen is reduced in OI Types I, II and IV (Sillence classification). Type III OI could not be distinguished from normal strains by the analysis used. From the collagen phenotype (biochemical parameters) we were able to distinguish different OI phenotypes and to correlate these with clinical phenotypes. One form of OI Type I produces an unstable collagen that is degraded to small peptides in culture.
| Identifer | oai:union.ndltd.org:LACETR/oai:collectionscanada.gc.ca:QMM.68562 |
| Date | January 1980 |
| Creators | Fraser, Judith. |
| Publisher | McGill University |
| Source Sets | Library and Archives Canada ETDs Repository / Centre d'archives des thèses électroniques de Bibliothèque et Archives Canada |
| Language | English |
| Detected Language | English |
| Type | Electronic Thesis or Dissertation |
| Format | application/pdf |
| Coverage | Doctor of Philosophy (Department of Biology) |
| Rights | All items in eScholarship@McGill are protected by copyright with all rights reserved unless otherwise indicated. |
| Relation | alephsysno: 000114587, proquestno: AAINK51955, Theses scanned by UMI/ProQuest. |
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