Congenital nephrotic syndrome in mice lacking CD2-associated protein

NY Shih, J Li, V Karpitskii, A Nguyen, ML Dustin… - Science, 1999 - science.org
NY Shih, J Li, V Karpitskii, A Nguyen, ML Dustin, O Kanagawa, JH Miner, AS Shaw
Science, 1999science.org
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 …
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.
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