The role of stem cells in fibrous dysplasia of bone and the Mccune-Albright syndrome.
Stem cells have become a major area of interest in the treatment of human disease, but more recently, stem cells have come to be appreciated as the cause of disease. Fibrous dysplasia of bone and the McCune-Albright Syndrome evolve from activating missense mutations in Gsalpha in pluripotent embryonic stem cells. The legacy of these mutations remains in a population of mutated multipotent post-natal skeletal stem cells ("mesenchymal" stem cells), which direct the formation of abnormal bone and a fibrotic marrow in fibrous dysplasia. Future therapeutic approaches for the treatment of fibrous dysplasia, the most significant component of the McCune-Albright Syndrome, will depend on a greater understanding of post-natal skeletal stem cell biology and how skeletal stem cells can be manipulated for efficient bone regeneration.
Robey
Pamela Gehron
PG
Craniofacial and Skeletal Diseases Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, DHHS, Bethesda, MD 20892, USA. probey@dir.nidcr.nih.gov
Kuznetsov
Sergei
S
Riminucci
Mara
M
Bianco
Paolo
P
D005357:Fibrous Dysplasia of Bone / Q000473:pathology*; D005359:Fibrous Dysplasia, Polyostotic / Q000473:pathology*; D006801:Humans; D059630:Mesenchymal Stem Cells; D039904:Pluripotent Stem Cells / Q000473:pathology*