Homology to NEPHRIN Lacking NEPH1, a Novel Protein with Proteinuria and Perinatal Lethality in Mice
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چکیده
10.1128/MCB.21.14.4829-4836.2001. 2001, 21(14):4829. DOI: Mol. Cell. Biol. Ramirez-Solis, Brian P. Zambrowicz and David R. Powell Michael Starbuck, Mary Brandt, Anupma Gupta, Ramiro Alexander Turner, Robert Geske, Charles A. Montgomery, C. Potter, Edith Hawkins, James P. Barrish, Brian N. Mathur, Dorit B. Donoviel, Deon D. Freed, Hannes Vogel, David G. Homology to NEPHRIN Lacking NEPH1, a Novel Protein with Proteinuria and Perinatal Lethality in Mice
منابع مشابه
Homodimerization and heterodimerization of the glomerular podocyte proteins nephrin and NEPH1.
Nephrin and NEPH1, the gene products of NPHS1 and NEPH1, are podocyte membrane proteins of the Ig superfamily. Similar to the nephrin knockout, mice lacking NEPH1 show severe proteinuria leading to perinatal death. To identify the ligand of NEPH1, the extracellular domain of NEPH1 was fused to human IgG. This NEPH1-Ig fusion protein labeled the glomerular capillary wall of mouse kidneys in a st...
متن کاملNEPH1 defines a novel family of podocin interacting proteins.
Mutations of NPHS1 or NPHS2, the genes encoding for the glomerular podocyte proteins nephrin and podocin, cause steroid-resistant proteinuria. In addition, mice lacking NEPH1 develop a nephrotic syndrome that resembles NPHS mutations, suggesting that all three proteins are essential for the integrity of glomerular podocytes. Podocin interacts with the C-terminal domain of nephrin and facilitate...
متن کاملNeph1 and nephrin interaction in the slit diaphragm is an important determinant of glomerular permeability.
Neph1-deficient mice develop nephrotic syndrome at birth, indicating the importance of this protein in the development of a normal glomerular filtration barrier. While the precise subcellular localization of Neph1 remains unknown, its relationship with other components of the glomerular filtration barrier is of great interest in this field. In this paper, we localize the expression of Neph1 to ...
متن کاملDissociation of NEPH1 from nephrin is involved in development of a rat model of focal segmental glomerulosclerosis.
Focal segmental glomerulosclerosis (FSGS) is a disease showing severe proteinuria, and the disease progresses to end-stage kidney failure in many cases. However, the pathogenic mechanism of FSGS is not well understood. The slit diaphragm (SD), which bridges the neighboring foot processes of glomerular epithelial cells, is understood to function as a barrier of the glomerular capillary wall. To ...
متن کاملNeph1 Is Reduced in Primary Focal Segmental Glomerulosclerosis, Minimal Change Nephrotic Syndrome, and Corresponding Experimental Animal Models of Adriamycin-Induced Nephropathy and Puromycin Aminonucleoside Nephrosis
BACKGROUND/AIMS The transmembrane proteins Neph1 and nephrin form a complex in the slit diaphragm (SD) of podocytes. As recent studies indicate an involvement of this complex in the polymerization of the actin cytoskeleton and proteinuria, we wanted to study the subcellular localization of Neph1 in the normal human kidney and its expression in focal segmental glomerulosclerosis (FSGS), minimal ...
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تاریخ انتشار 2001