Bovine osteogenesis imperfecta (Australia type): Morphological characterization of an animal model for osteogenesis imperfecta in man (genetics, bone, veterinary, collagen, pathology) [microform]
- Bib ID:
- 3287563
- Format:
- Book and Microform
- Author:
- Denholm, Laurence John
- Description:
- 360 p.
- Summary:
-
Ultrastructural lesions typical of osteogenesis imperfecta and dentinogenesis imperfecta in man were demonstrated in an experimentally reproducible, autosomal dominant syndrome of congenital bone fragility, translucent teeth, blue sclerae and hypoplastic tendons in 44% of singleton progeny of a normal Holstein-Friesian bull. Decreased connective tissue matrix : cell ratios and collagen fibril volumetric densities were caused by depressed fibril lateral growth, restricted to tissues which normally develop wide fibril size distributions, and increasing in severity with fetal age. Fibril attenuation was not simply proportional to fibril growth since thick fibrils were not attenuated in tissues with more uniform diameter size distributions.
Fibril attenuation was spatially and temporally related to cell degeneration, most prominent during fetal odontogenesis where autophagy, apoptosis and necrosis of odontoblasts, and attenuation of predentin fibrils followed dilation of rough endoplasmic reticular cisternae by dense, granular material. Osteopenia was not present at birth because neo-osseous porosis from a depressed cellular rate of matrix apposition was compensated during fetal development by prolonged bone formation on apposition surfaces and reduced endosteal bone resorption, with secondary congenital hypocalcitriolemia and parathyroid hypoplasia in severe cases.
Decreased ultimate deflection in biomechanical testing of cortical bone cores, decreased hydroxyapatite crystallinity by X-ray diffractometry, decreased lamellar bone, dentin and tendon birefringence, reduced tissue content of bone small proteoglycan, post-translational overmodification of five genetically distinct collagen peptides and non-uniform collagen fibril attenuation suggest a complex defect of matrix supramolecular organization and cell degeneration, with several features unlike those reported in type I collagen gene mutations. A lower than expected incidence of disease-discordant twins suggests that parabiosis by placental fusion in bovine multiple pregnancies may facilitate normal development in genetically abnormal BOI(A) calves. These studies established BOI(A) as a valid, reproducible animal model, and human cases of O.I. with similar multiple, post-translational collagen defects have since been identified.
- Notes:
-
- (UnM)AAI8517021
- Source: Dissertation Abstracts International, Volume: 46-07, Section: B, page: 2227.
- Thesis (Ph.D.)--Cornell University, 1985.
- Reproduction:
- Microfiche. Ann Arbor, Mich.: University Microfilms International.
- Subject:
- Biology, Veterinary Science
- Other authors/contributors:
- Cornell University
- Copyright:
-
In Copyright
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Copyright status was determined using the following information:
- Material type:
- Literary Dramatic Musical
- Published status:
- Unpublished
- Creation date:
- 1985
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