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Deletion of RBP-Jkappa gene in mesenchymal cells causes rickets like symptoms in the mouse

Yan Gao1, Jemma Victoria Walker1, Christopher Tredwin1

  • 1Stem Cells & Regenerative Medicine Laboratory, Peninsula Dental School, Faculty of Health, University of Plymouth, 16 Research Way, Plymouth, PL6 8BU UK.

Current Medicine (Cham, Switzerland)
|June 13, 2022
PubMed

Insights

Notch and Wnt5a pathways are crucial for skeletal development. Deleting RBP-Jkappa in mesenchymal cells impairs Wnt5a signaling, causing Rickets-like symptoms that can be rescued by Wnt5a.

Area of Science:

  • Molecular Biology
  • Developmental Biology
  • Genetics

Background:

  • The Notch pathway, mediated by RBP-Jkappa, and the Wnt signaling pathway are critical in cellular development.
  • Previous research identified RBP-Jkappa's regulation of Wnt5a in dermal papilla cells.

Purpose of the Study:

  • To investigate the role of RBP-Jkappa in regulating Wnt5a in chondrocytes and osteoblasts.
  • To explore the connection between Notch and Wnt5a pathways in skeletal development and Rickets.

Main Methods:

  • Conditional mesenchymal deletion of RBP-Jkappa in mice.
  • Molecular analysis of Wnt5a expression at mRNA and protein levels.
  • Assessment of chondrocyte and osteoblast differentiation.
  • Rescue experiments using recombinant Wnt5a treatment.

Main Results:

  • Mice lacking mesenchymal RBP-Jkappa exhibited Rickets-like symptoms.
  • These mice showed reduced Wnt5a expression in chondrocytes and osteoblasts, impairing differentiation.
  • Recombinant Wnt5a treatment rescued cellular and tissue-level defects.

Conclusions:

  • RBP-Jkappa directly regulates Wnt5a expression in chondrocytes and osteoblasts.
  • This Notch-Wnt5a axis is essential for skeletal development.
  • Dysregulation of this axis contributes to Rickets-like conditions.
Keywords:
BoneCartilageNotchRicketsWnt5a

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