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The Hedgehog gene (Hh) was first discovered due to its control of the growth of disorganized, hair-like bristles phenotype in Drosophila, much like hedgehog spines. Hh plays a crucial role in the development of organs and the maintenance of homeostasis in both invertebrates and vertebrates. However, while Drosophila has only one Hh protein, mammals have multiple functional Hedgehog proteins - Sonic (Shh), Desert (Dhh), and Indian Hedgehog (Ihh). All of these homologous proteins have adapted to...
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The endocrine system produces and secretes hormones, which interact with the skeletal system. These hormones control bone growth, maintain bone once it is formed, and remodel it.
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Stimulation of Notch Signaling in Mouse Osteoclast Precursors
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Hedgehog Activation Regulates Human Osteoblastogenesis.

Shoko Onodera1, Akiko Saito2, Hironori Hojo3

  • 1Department of Biochemistry, Tokyo Dental College, Tokyo 101-0061, Japan; Center for Disease Biology and Integrative Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo 113-8656, Japan.

Stem Cell Reports
|June 13, 2020
PubMed
Summary
This summary is machine-generated.

Hedgehog (Hh) signaling regulates bone formation. This study used stem cells to show Hh activation promotes bone growth in Gorlin syndrome and corrects poor bone formation in McCune-Albright syndrome (MAS), suggesting new therapeutic targets.

Keywords:
Gorlin syndromeMcCune-Albright syndromecalcificationfibrous dysplasiahedgehog pathwayosteogenesispatient-specific iPSC

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Area of Science:

  • Stem cell biology
  • Genetics
  • Bone biology

Background:

  • Gorlin syndrome and McCune-Albright syndrome (MAS) exhibit opposing bone phenotypes.
  • Hedgehog (Hh) signaling activity differs between these genetic conditions.

Purpose of the Study:

  • To investigate the role of Hh signaling in osteogenesis using patient-derived stem cells.
  • To understand how Hh pathway dysregulation contributes to bone abnormalities in Gorlin syndrome and MAS.

Main Methods:

  • Generation of human induced pluripotent stem cell (iPSC)-based disease models for Gorlin syndrome and MAS.
  • Assessment of osteoblastogenesis and mineralization in vitro.
  • Pharmacological modulation of Hh signaling activity.

Main Results:

  • Gorlin syndrome iPSCs displayed enhanced osteoblastogenesis and mineralization with activated Hh signaling.
  • MAS iPSCs showed impaired mineralization, which was rescued by Hh signaling activation.
  • Upregulation of specific transcription factors was observed in Gorlin syndrome osteoblasts.

Conclusions:

  • Hh signaling is a critical regulator of osteoblast differentiation from precursor cells.
  • Targeting Hh signaling may offer novel therapeutic strategies for genetic calcification disorders.