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ZEB2 protein is vital for ureter development – ZEB2 mutations underlie urinary tract dysfunction in Mowat-Wilson syndrome

23 April 2026

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If an escalator stops working, passive passengers will accumulate both on the stairs and in a disgruntled cluster at the start. This is what happens if the ureter connecting kidney and bladder cannot effectively move urine through the system. The backlog in both ureter and kidney is characteristic of Mowat-Wilson syndrome, a rare genetic disorder caused by mutations in the ZEB2 gene that causes a wide range of developmental defects. To understand what role ZEB2 plays in ureter development, researchers studied genetically engineered mice in which the gene was deleted in ureter precursor cells. In these mice, cells that normally become smooth muscle cells (red in the mouse ureter section, left) fail to develop (right). Without this muscle layer, the ureter can’t contract to move urine, causing swelling of the ureter and kidney and ultimately obstruction, revealing a key molecular mechanism underlying urinary tract anomalies in Mowat-Wilson syndrome patients.

Written by Anthony Lewis

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