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Related Experiment Videos

Dentine is biochemically abnormal in osteogenesis imperfecta.

J P Gage, M J Francis, G E Whitaker

    Clinical Science (London, England : 1979)
    |April 1, 1986
    PubMed
    Summary

    Osteogenesis imperfecta, a brittle bone disease, often shows normal teeth despite sharing type I collagen. Biochemical analysis revealed frequent abnormalities in dentine collagen composition in patients, challenging current classifications.

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    Area of Science:

    • Biochemistry
    • Genetics
    • Oral Biology
    • Orthopedics

    Background:

    • Osteogenesis imperfecta (OI) is characterized by brittle bones due to defects in type I collagen.
    • Dentine, like bone, contains type I collagen, yet teeth in OI patients can appear clinically normal, presenting a paradox.
    • Understanding dentine collagen in OI is crucial for a comprehensive view of the disorder.

    Purpose of the Study:

    • To investigate the amino acid composition of insoluble dentine collagen in osteogenesis imperfecta patients.
    • To identify biochemical differences in dentine collagen between control subjects and OI patients.
    • To assess the clinical relevance of dentine collagen abnormalities in OI classification.

    Main Methods:

    • Amino acid analysis of insoluble dentine collagen.

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  • Samples obtained from 16 deciduous and 18 permanent teeth from control subjects.
  • Samples obtained from 59 patients with various forms of osteogenesis imperfecta.

    • Main Results:

    • Significant differences in amino acid composition were observed in 55 out of 59 patient samples compared to controls.
    • Key differences included alterations in the number of lysine residues.
    • The relative amounts of hydroxylysine to lysine were also significantly different in patients.

    Conclusions:

    • Biochemical abnormalities in dentine collagen are highly frequent in osteogenesis imperfecta.
    • These findings highlight the systemic impact of OI beyond bone fragility.
    • Current classifications of OI based on clinical presentation of dentinogenesis imperfecta may be unreliable.