Document Detail

Collagen defect of bone in osteogenesis imperfecta (Type I). An electron microscopic study.
MedLine Citation:
PMID:  6697589     Owner:  NLM     Status:  MEDLINE    
Osteogenesis imperfecta (OI) is an inherited disorder of connective tissue metabolism characterized by fragility of the bones, resulting in multiple fractures. In this study electron microscopic examination of the osteoid region of iliac crest bone biopsy specimens from a 55-year-old man and his three sons (18, 21, and 25 years of age) affected with the common Type I (autosomal dominant) form of OI revealed collagen fibrils consistently smaller in diameter than those from normal age-matched control subjects, with the majority of OI fibrils measuring 0.04-0.06 micron, as opposed to 0.06-0.08 micron in the control subjects. This defect may be due to the biosynthesis of Type III collagen, not normally found in adult lamellar bone or, more likely, to a failure of maturation of Type I fibrils to their normal diameters, possibly owing to the increased levels of hydroxylysine previously reported in OI collagen, a factor known to be inversely related to fibril diameter.
C J Jones; C Cummings; J Ball; P Beighton
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Clinical orthopaedics and related research     Volume:  -     ISSN:  0009-921X     ISO Abbreviation:  Clin. Orthop. Relat. Res.     Publication Date:  1984 Mar 
Date Detail:
Created Date:  1984-04-04     Completed Date:  1984-04-04     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0075674     Medline TA:  Clin Orthop Relat Res     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  208-14     Citation Subset:  AIM; IM    
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MeSH Terms
Collagen / biosynthesis*
Genes, Dominant
Ilium / pathology
Microscopy, Electron
Middle Aged
Osteogenesis Imperfecta / genetics,  pathology*
Reg. No./Substance:

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