Ablation of Enpp6 results in transient bone hypomineralization

Scott Dillon, Karla Oldknow, Shun-Neng Hsu, Louise Stephen, Rongling Wang, William P Cawthorn, Alan J. Stewart, Fabio Nudelman, Nicholas M Morton, Colin Farquharson

Research output: Contribution to journalArticlepeer-review

Abstract / Description of output

Biomineralization is a fundamental process key to the development of the skeleton. The phosphatase orphan phosphatase 1 (PHOSPHO1), which likely functions within extracellular matrix vesicles, has emerged as a critical regulator of biomineralization. The biochemical pathways which generate intravesicular PHOSPHO1 substrates are however currently unknown. We hypothesized that the enzyme ectonucleotide pyrophosphatase/phosphodiesterase (ENPP6) is an upstream source of PHOSPHO1 substrate. To test this, we characterized skeletal phenotypes of mice homozygous for a targeted deletion of Enpp6 (Enpp6‒/‒). Micro-computed tomography of the trabecular compartment revealed transient hypomineralization in Enpp6‒/‒ tibiae (p < 0.05) that normalized by 12 weeks of age. Whole-bone cortical analysis also revealed significantly hypomineralized proximal bone in 4– but not 12–week old Enpp6‒/‒ mice (p < 0.05) compared to wild-type animals. Backscattered scanning electron microscopy revealed a failure in 4-week-old trabecular bone
Original languageEnglish
JournalJBMR Plus
Early online date24 Nov 2020
Publication statusE-pub ahead of print - 24 Nov 2020

Keywords / Materials (for Non-textual outputs)

  • ENPP6
  • Matrix mineralization
  • Osteoblast
  • Matrix vesicle


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