Upper urinary tract pacemaker cells join the GLI club.
Level V
Where this comes from
- Record sourced from PubMed, PMID 21339639.
- Also identified by DOI 10.1172/JCI46400 and PMC identifier 3049378.
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Abstract
Mutations in GLI3, a component of the Sonic Hedgehog (Shh) signaling pathway, cause a variety of human developmental syndromes. In this issue of the JCI, Cain and colleagues show that tightly regulated GLI3 repressor activity is essential for Shh-dependent differentiation of upper urinary tract pacemaker cells and the efficient flow of urine from the kidney to the bladder. These results link defective pacemaker cell differentiation with hydronephrosis and provide a cellular basis for one of the abnormal renal defects observed in humans with the GLI3-linked disease Pallister-Hall syndrome.
Medical subject headings
- Kruppel-Like Transcription Factors
- Nerve Tissue Proteins
- Urinary Tract