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WT1 Activates a Glomerular-Specific Enhancer Identified from the Human Nephrin Gene

Gordon Guo^*, Debra J. Morrison, Jonathan D. Licht and Susan E. Quaggin^*,

*Samuel Lunenfeld Research Institute, Mt. Sinai Hospital, University of Toronto, Toronto, Ontario, Canada; Division of Hematology/Oncology, Department of Medicine, Mount Sinai School of Medicine, New York, New York; Divison of Nephrology, St. Michael’s Hospital, Toronto, Ontario, Canada.

Correspondence to Dr. Susan E. Quaggin, Samuel Lunenfeld Research Institute #855, Mount Sinai Hospital, 600 University Ave., Toronto, Ontario M5G 1X5, Canada. Phone: 416-586-4800 ext. 2859; Fax: 416-586-8588; E-mail: quaggin{at}mshri.on.ca

The glomerular filtration barrier separates the blood from the urinary space. Nephrin is a transmembrane protein that belongs to the immunoglobulin superfamily and is localized to the slit diaphragms that are a critical component of this filtration barrier. Mutations in the nephrin gene (NPHS1) lead to congenital Finnish nephropathy, whereas alterations in the level of nephrin expression have been identified in a wide range of acquired glomerular diseases. A 186-bp fragment from the human NPHS1 promoter is capable of directing podocyte-specific expression of a -galactosidase transgene when placed in front of a heterologous minimal promoter in transgenic mice. The Wilms tumor suppressor gene (WT1) is a zinc-finger–containing transcription factor that is coexpressed with NPHS1 in differentiated podocytes; gel shift binding assays demonstrate that a recombinant WT1 protein can bind and activate the 186-bp NPHS1 fragment in a sequence-specific manner. Taken together, these results suggest that WT1 may be required for regulation of the NPHS1 gene in vivo.

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