1. Bone matrix hypermineralization in prolyl-3 hydroxylase 1 deficient mice
- Author
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Hans Peter Bächinger, Frank Rauch, Paul Roschger, Klaus Klaushofer, Janice A. Vranka, and Nadja Fratzl-Zelman
- Subjects
musculoskeletal diseases ,0301 basic medicine ,Pathology ,medicine.medical_specialty ,Histology ,Genotype ,Physiology ,Endocrinology, Diabetes and Metabolism ,Procollagen-Proline Dioxygenase ,Bone Matrix ,Matrix (biology) ,Bone tissue ,Bone and Bones ,Extracellular matrix ,03 medical and health sciences ,Mice ,Calcification, Physiologic ,Osteoclast ,Bone Density ,medicine ,Animals ,Analysis of Variance ,Osteoid ,Chemistry ,Osteoblast ,medicine.disease ,030104 developmental biology ,medicine.anatomical_structure ,Osteogenesis imperfecta ,Cancellous bone - Abstract
Lack of prolyl 3-hydroxylase 1 (P3H1) due to mutations in P3H1 results in severe forms of recessive osteogenesis imperfecta. In the present study, we investigated the bone tissue characteristics of P3H1 null mice. Histomorphometric analyses of cancellous bone in the proximal tibia and lumbar vertebra in 1-month and 3-month old mice demonstrated that P3H1 deficient mice had low trabecular bone volume and low mineral apposition rate, but normal osteoid maturation time and normal osteoblast and osteoclast surfaces. Quantitative backscattered electron imaging revealed that the bone mineralization density distribution was shifted towards higher values, indicating hypermineralization of bone matrix. It thus appears that P3H1 deficiency leads to decreased deposition of extracellular matrix by osteoblasts and increased incorporation of mineral into the matrix.
- Published
- 2015