Bone matrix hypermineralization in prolyl-3 hydroxylase 1 deficient mice

Nadja Fratzl-Zelman, Hans Peter Bächinger, Janice Vranka, Paul Roschger, Klaus Klaushofer, Frank Rauch

Research output: Contribution to journalArticle

6 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)15-22
Number of pages8
JournalBone
Volume85
DOIs
StatePublished - Apr 1 2016

Fingerprint

Bone Matrix
Osteoblasts
Minerals
Osteogenesis Imperfecta
Physiologic Calcification
Lumbar Vertebrae
Osteoclasts
Tibia
Bone Density
Extracellular Matrix
2-oxoglutarate 3-dioxygenase proline
Electrons
Bone and Bones
Mutation

Keywords

  • Bone histomorphometry
  • Bone mineralization density distribution
  • Murine bone
  • Prolyl-3 hydroxylase-1 deficiency
  • Quantitative backscattered electron imaging

ASJC Scopus subject areas

  • Physiology
  • Endocrinology, Diabetes and Metabolism
  • Histology

Cite this

Fratzl-Zelman, N., Bächinger, H. P., Vranka, J., Roschger, P., Klaushofer, K., & Rauch, F. (2016). Bone matrix hypermineralization in prolyl-3 hydroxylase 1 deficient mice. Bone, 85, 15-22. https://doi.org/10.1016/j.bone.2016.01.018

Bone matrix hypermineralization in prolyl-3 hydroxylase 1 deficient mice. / Fratzl-Zelman, Nadja; Bächinger, Hans Peter; Vranka, Janice; Roschger, Paul; Klaushofer, Klaus; Rauch, Frank.

In: Bone, Vol. 85, 01.04.2016, p. 15-22.

Research output: Contribution to journalArticle

Fratzl-Zelman, N, Bächinger, HP, Vranka, J, Roschger, P, Klaushofer, K & Rauch, F 2016, 'Bone matrix hypermineralization in prolyl-3 hydroxylase 1 deficient mice', Bone, vol. 85, pp. 15-22. https://doi.org/10.1016/j.bone.2016.01.018
Fratzl-Zelman N, Bächinger HP, Vranka J, Roschger P, Klaushofer K, Rauch F. Bone matrix hypermineralization in prolyl-3 hydroxylase 1 deficient mice. Bone. 2016 Apr 1;85:15-22. https://doi.org/10.1016/j.bone.2016.01.018
Fratzl-Zelman, Nadja ; Bächinger, Hans Peter ; Vranka, Janice ; Roschger, Paul ; Klaushofer, Klaus ; Rauch, Frank. / Bone matrix hypermineralization in prolyl-3 hydroxylase 1 deficient mice. In: Bone. 2016 ; Vol. 85. pp. 15-22.
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