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Related Experiment Videos

Hematopoietic stem-cell contribution to ectopic skeletogenesis.

Frederick S Kaplan1, David L Glaser, Eileen M Shore

  • 1Center for Research in Fibrodyplasia Ossificans Progressiva and Related Disorders, Department of Orthopaedic Surgery, University of Pennsylvania School of Medicine, Philadelphia, PA 19104-6081, USA. frederick.kaplan@uphs.upenn.edu

The Journal of Bone and Joint Surgery. American Volume
|February 3, 2007
PubMed
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Bone marrow transplantation did not cure fibrodysplasia ossificans progressiva (FOP). Immunosuppression, not hematopoietic cells, appeared to reduce ectopic skeletogenesis in a patient with FOP.

Area of Science:

  • Genetics and Regenerative Medicine
  • Skeletal Biology
  • Immunology

Background:

  • Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by ectopic skeletogenesis due to dysregulated bone morphogenetic protein (BMP) signaling.
  • Hematopoietic cells have been investigated for their potential role in FOP pathogenesis and as a basis for curative therapies.

Observation:

  • A patient with FOP who received a bone marrow transplant 25 years prior for aplastic anemia was studied to assess the impact of hematopoietic cell replacement and immunosuppression on FOP progression.
  • Murine models involving transplantation of hematopoietic stem cells and BMP4-induced heterotopic ossification were used to delineate the contribution of hematopoietic cells to ectopic bone formation.

Findings:

  • Bone marrow transplantation alone did not prevent ectopic skeletogenesis in the FOP patient.

Related Experiment Videos

  • Pharmacologic immunosuppression following transplantation modulated FOP activity and reduced skeletal ectopia.
  • In murine models, hematopoietic cells contributed to early and late stages of BMP4-induced heterotopic ossification but not to the ossification process itself.
  • Both BMP4 induction and FOP-associated BMP signaling dysregulation recruit hematopoietic and non-hematopoietic cells to form ectopic bone.

    • Implications:

    • The hematopoietic cell population is unlikely to be the primary site of intrinsic BMP signaling dysregulation in FOP.
    • Immunosuppression may ameliorate ectopic skeletogenesis in genetically susceptible individuals with FOP.
    • Hematopoietic cells can contribute to ectopic skeleton formation but are insufficient to initiate the process independently.