Congenital nephrotic syndrome in mice lacking CD2-associated protein.
CD2-associated protein (CD2AP) is an 80-kilodalton protein that is critical for stabilizing contacts between T cells and antigen-presenting cells. In CD2AP-deficient mice, immune function was compromised, but the mice died at 6 to 7 weeks of age from renal failure. In the kidney, CD2AP was expressed primarily in glomerular epithelial cells. Knockout mice exhibited defects in epithelial cell foot processes, accompanied by mesangial cell hyperplasia and extracellular matrix deposition. Supporting a role for CD2AP in the specialized cell junction known as the slit diaphragm, CD2AP associated with nephrin, the primary component of the slit diaphragm.
Shih
N Y
NY
Center for Immunology and Department of Pathology, Washington University, Saint Louis, MO 63110, USA.
Li
J
J
Karpitskii
V
V
Nguyen
A
A
Dustin
M L
ML
Kanagawa
O
O
Miner
J H
JH
Shaw
A S
AS
D048868:Adaptor Proteins, Signal Transducing; D000818:Animals; D001485:Basement Membrane / Q000648:ultrastructure; D003598:Cytoskeletal Proteins; D004847:Epithelial Cells / Q000378:metabolism / Q000648:ultrastructure; D016326:Extracellular Matrix Proteins / Q000378:metabolism; D005920:Glomerular Mesangium / Q000378:metabolism / Q000648:ultrastructure; D007365:Intercellular Junctions / Q000378:metabolism / Q000648:ultrastructure; D007678:Kidney Glomerulus / Q000098:blood supply / Q000378:metabolism* / Q000648:ultrastructure*; D008213:Lymphocyte Activation; D008565:Membrane Proteins; D051379:Mice; D018345:Mice, Knockout; D008854:Microscopy, Electron; D009404:Nephrotic Syndrome / Q000151:congenital* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D011506:Proteins / Q000235:genetics / Q000378:metabolism*; D011993:Recombinant Fusion Proteins / Q000378:metabolism; D013601:T-Lymphocytes / Q000276:immunology
D048868:Adaptor Proteins, Signal Transducing; C114545:CD2-associated protein; D003598:Cytoskeletal Proteins; D016326:Extracellular Matrix Proteins; D008565:Membrane Proteins; D011506:Proteins; D011993:Recombinant Fusion Proteins; C113267:nephrin