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Isolation and Culture of Cells from the Nephrogenic Zone of the Embryonic Mouse Kidney
Published on: April 22, 2011
SOX9 controls epithelial branching by activating RET effector genes during kidney development
Antoine Reginensi1, Michael Clarkson, Yasmine Neirijnck
1INSERM U636, F-06108 Nice, France.
Human Molecular Genetics
|January 8, 2011
Summary
SOX9 and SOX8 are crucial for kidney development by regulating the GDNF/RET signaling pathway. These genes are essential for ureter branching and maintaining kidney structure, explaining defects in campomelic dysplasia.
Area of Science:
- Developmental biology
- Genetics
- Molecular biology
Background:
- Congenital kidney and urinary tract abnormalities are common in newborns.
- The GDNF/RET signaling pathway controls kidney growth, but downstream molecular mechanisms are unclear.
Purpose of the Study:
- To investigate the role of SOX9 and SOX8 in kidney development and RET signaling.
- To understand the molecular basis of kidney defects in campomelic dysplasia.
Main Methods:
- Tissue-specific knockout in mice.
- Gene expression analysis.
- Genetic analysis of RET downstream targets.
Main Results:
- SOX8 and SOX9 are essential for ureter branching and kidney development.
- SOX8/9 act downstream of GDNF signaling to activate RET effector genes.
- SOX9 maintains ureteric tip identity and prevents ectopic nephron formation.
Conclusions:
- SOX9 and SOX8 are key regulators of kidney organogenesis via the RET pathway.
- These findings elucidate the cause of kidney hypoplasia in campomelic dysplasia patients.
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