Bone matrix hypermineralization in prolyl-3 hydroxylase 1 deficient mice.

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.

• Authors: Nadja Fratzl-Zelman, Hans-Peter Bächinger, Janice A Vranka, Paul Roschger, Klaus Klaushofer, Frank Rauch
• Journal: Bone (Bone) Vol. 85 Pg. 15-22 (Apr 2016) ISSN: 1873-2763 [Electronic] United States
• PMID: 26808442 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2016 Elsevier Inc. All rights reserved.

Chemical References

• Procollagen-Proline Dioxygenase
• proline, 2-oxoglutarate 3-dioxygenase

Topics

• Analysis of Variance
• Animals
• Bone Density
• Bone Matrix (physiology)
• Bone and Bones (metabolism, pathology)
• Calcification, Physiologic (physiology)
• Genotype
• Mice
• Procollagen-Proline Dioxygenase (deficiency, metabolism)