Cbfa1, a candidate gene for cleidocranial dysplasia syndrome, is essential for osteoblast differentiation and bone development

Article Abstract:

The role of Cbfa1 genes in the progression of bone differentiation and osteogenesis was analyzed in Cbfa1-deficient mice. Southern blot analysis of the Cbfa1-mutant mice indicated the presence of inhibited osteoblast development from the mesenchyme. Mutations in the Cbfa1 gene also prevented bone formation during the early stages of ossification. On the other hand, heterozygous Cbfa1 mutant mice exhibited abnormal skeletal development that was similar to the symptoms of cleidocranial dysplasia syndrome.

author: Olsen, Bjorn R., Owen, Michael J., Beddington, Rosa S.P., Stamp, Gordon W.H., Mundlos, Stefan, Crompton, Tessa, Gilmour, Kimberly C., Otto, Florian, Thornell, Anders P., Denzel, Angela, Rosewell, Ian R., Selby, Paul B.
Developmental genetics

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Targeted disruption of Cbfa1 results in a complete lack of bone formation owing to maturational arrest of osteoblasts

Article Abstract:

The biochemical functions of the Cbfa1 core-binding factor were characterized in fetal mice with mutated Cbfa1 genes. The mutated mice died after birth due to a complete lack of bone formation that was induced by the disruption of the Cbfa1 gene. Histological analysis of the skeleton of the mice indicated the presence of low levels of osteopontin and osteolacin. Furthermore, maturational arrest occured during the early stage of osteoblast differentiation in the mutated mice embryos.

author: Kishimoto, T., Sasaki, K., Kitamura, Y., Bronson, R.T., Sato, M., Yamaguchi, A., Komori, T., Yagi, H., Nomura, S., Deguchi, K., Shimizu, Y., Gao, Y.-H., Inada, M., Okamoto, R., Yoshiki, S.

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The MAP kinase pathway controls differentiation from double-negative to double-positive thymocyte

Article Abstract:

Maturation and proliferation of and antigen receptor gene rearrangement in T cells are coordinated at two major control points. The expression of different forms of the T cell receptor influences progression through these control points. Studies show that the differentiation of immature thymocytes from double-negative to double-positive cells is regulated by a mitogen-activated protein kinase.

author: Owen, Michael J., Crompton, Tessa, Gilmour, Kimberly C.
Protein kinases, Cell receptors, T cells

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subjects list: Research, Bones, Genetic aspects, Growth, Cell differentiation, Bone development
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