Congenital Nephrotic Syndrome in Mice Lacking CD2-Associated Protein
Neng-Yao Shih,
1*
Jun Li,
1*
Vladimir Karpitskii,
1
Ancho Nguyen,
1
Michael L. Dustin,
1
Osami Kanagawa,
1
Jeffrey H. Miner,
2
Andrey S. Shaw
1
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.
1 Center for Immunology and Department of
Pathology,
2 Department of Medicine, Renal Division,
Washington University, Saint Louis, MO 63110, USA.
*
These authors contributed equally to this work.
To whom correspondence should be addressed. E-mail:
shaw{at}immunology.wustl.edu
