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Frizzled 9 knock-out mice have abnormal B-cell development.

Blood | Mar 15, 2005

http://www.ncbi.nlm.nih.gov/pubmed/15572594

The binding of frizzled (Fzd) receptors by their Wnt ligands results in the inhibition of beta-catenin degradation and subsequent transcription of beta-catenin/LEF-inducible genes. The beta-catenin pathway is known to be involved in development, tumorigenesis, and stem cell self-renewal. In humans, the FZD9 gene lies in the region of chromosome 7q11.23 deleted in the neurodevelopmental disorder, Williams-Beuren syndrome (WBS). Fzd9-/- mice show no obvious features of WBS, but reveal a role for Fzd9 in lymphoid development and maturation. Fzd9-/- mice show pronounced splenomegaly, thymic atrophy, and lymphadenopathy with age, with accumulation of plasma cells in lymph nodes. There is a depletion of developing B cells in the bone marrow (BM), particularly in the pre-B stage where immunoglobulin heavy chains are expressed and the cells are undergoing clonal expansion prior to light chain rearrangement. The pre-B defect is partially intrinsic to the hematopoietic system; as in competitive BM reconstitution studies, Fzd9-/- -derived BM exhibits defective B-cell development when implanted into a wild-type host. Mature B cells are present in normal numbers in lymph node and spleen. These findings suggest a role for Fzd9 signaling in lymphoid development, particularly at points where B cells undergo self-renewal prior to further differentiation.

Pubmed ID: 15572594 RIS Download

Mesh terms: Animals | Atrophy | B-Lymphocytes | Bone Marrow Cells | Bone Marrow Transplantation | Cell Differentiation | Chromosomes, Human, Pair 7 | Humans | Immunoglobulin Light Chains | Lymph Nodes | Lymphatic Diseases | Lymphopoiesis | Mice | Mice, Knockout | Receptors, Neurotransmitter | Signal Transduction | Somatic Hypermutation, Immunoglobulin | Spleen | TCF Transcription Factors | Thymus Gland | Williams Syndrome | beta Catenin

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Associated grants

  • Agency: PHS HHS, Id: K08-90450
  • Agency: NICHD NIH HHS, Id: R01 HD39921
  • Agency: NIGMS NIH HHS, Id: T32 GM08748

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