Congenital nephrotic syndrome in mice lacking CD2-associated protein

Neng Yao Shih, Jun Li, Vladimir Karpitskii, Ancho Nguyen, Michael L. Dustin, Osami Kanagawa, Jeffrey H. Miner, Andrey S. Shaw

Research output: Contribution to journalArticlepeer-review

246 Scopus citations


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 unction known as the slit diaphragm, CD2AP associated with nephrin, the primary component of the slit diaphragm.

Original languageEnglish
Pages (from-to)312-315
Number of pages4
Issue number5438
StatePublished - Oct 8 1999


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