Bone matrix hypermineralization in prolyl-3 hydroxylase 1 deficient mice

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

Research output: Contribution to journalArticlepeer-review

14 Scopus citations


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
StatePublished - Apr 1 2016


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

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Histology
  • Physiology


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