title: Upper urinary tract pacemaker cells join the GLI club.
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.
authors.affiliation: Department of Physiology and Biophysics, Cornell University Medical College, New York, New York 10021-4805, USA. daherzli@med.cornell.edu
authors.forename: Doris
authors.initials: D
authors.lastname: Herzlinger
mesh_terms: D000818:Animals; D002454:Cell Differentiation; D004594:Electrophysiology; D018507:Gene Expression Regulation, Developmental; D053823:Hedgehog Proteins / Q000378:metabolism; D006801:Humans; D007668:Kidney / Q000033:anatomy & histology; D051741:Kruppel-Like Transcription Factors / Q000502:physiology*; D008954:Models, Biological; D009119:Muscle Contraction; D009130:Muscle, Smooth / Q000473:pathology; D009419:Nerve Tissue Proteins / Q000502:physiology*; D054975:Pallister-Hall Syndrome / Q000378:metabolism; D001743:Urinary Bladder / Q000033:anatomy & histology; D014551:Urinary Tract / Q000033:anatomy & histology* / Q000196:embryology*; D000074290:Zinc Finger Protein Gli3
chemical_list: C065683:GLI3 protein, human; D053823:Hedgehog Proteins; D051741:Kruppel-Like Transcription Factors; D009419:Nerve Tissue Proteins; C509239:SHH protein, human; D000074290:Zinc Finger Protein Gli3