Article
Demonstration of an osteoblast defect in two cases of human malignant osteopetrosis. Correction of the phenotype after bone marrow transplant.
The Journal of clinical investigation - 15 Oct 1996
Lajeunesse D, Busque L, Ménard P, Brunette M G, Bonny Y
Abstract excerpt
Osteopetrosis is an inherited disorder characterized by bone sclerosis due to reduced bone resorption. Here we report that human osteopetrotic osteoblast-like (Ob) cells express a defective phenotype in primary cultures in vitro, and that bone marrow transplant (BMT) corrects osteoblast function....
Topics
- Alkaline Phosphatase
- Bone Marrow Transplantation
- Calcitriol
- Cells, Cultured
- Female
- Genetic Linkage
- Humans
- Infant
- Macrophage Colony-Stimulating Factor
- Osteoblasts
- Osteocalcin
- Osteopetrosis
- Phenotype
- Polymerase Chain Reaction
- Receptors, Androgen
- X Chromosome