期刊
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
卷 24, 期 23, 页码 -出版社
MDPI
DOI: 10.3390/ijms242317010
关键词
osteogenesis imperfecta; bone; OIM; collagen; osteocyte lacunae
This study investigates the organization of collagen and osteocyte lacunae in the long bones of mice with osteogenesis imperfecta (OI), a rare congenital bone disorder. The findings suggest that the alterations in collagen matrix and osteocyte lacunae organization contribute to bone fragility.
Osteogenesis imperfecta (OI) is a rare congenital bone dysplasia generally caused by a mutation of one of the type I collagen genes and characterized by low bone mass, numerous fractures, and bone deformities. The collagen organization and osteocyte lacuna arrangement were investigated in the long bones of 17-week-old wildtype (WT, n = 17) and osteogenesis imperfecta mice (OIM, n = 16) that is a validated model of severe human OI in order to assess their possible role in bone fragility. Fractures were counted after in vivo scanning at weeks 5, 11, and 17. Humerus, femur, and tibia diaphyses from both groups were analyzed ex vivo with pQCT, polarized and ordinary light histology, and Nano-CT. The fractures observed in the OIM were more numerous in the humerus and femur than in the tibia, whereas the quantitative bone parameters were altered in different ways among these bones. Collagen fiber organization appeared disrupted, with a lower birefringence in OIM than WT bones, whereas the osteocyte lacunae were more numerous, more spherical, and not aligned in a lamellar pattern. These modifications, which are typical of immature and less mechanically competent bone, attest to the reciprocal alteration of collagen matrix and osteocyte lacuna organization in the OIM, thereby contributing to bone fragility.
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