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A Humanized Mouse Model of Idiopathic Nephrotic Syndrome Suggests a Pathogenic Role for Immature Cells

Anne-Laure Sellier-Leclerc^*, Arnaud Duval, Stéphanie Riveron^*, Marie-Alice Macher, Georges Deschenes, Chantal Loirat, Marie-Christine Verpont, Michel Peuchmaur^||, Pierre Ronco, Renato C. Monteiro^* and Elie Haddad

^* INSERM U699, Faculté de Médecine Denis Diderot, Université Paris 7; Service de Néphrologie and ^|| Service de Pathologie, EA 3102, Hôpital Robert Debré, AP-HP, and Université Paris 7, and INSERM UMR S 702, Université Pierre et Marie Curie-Paris 6, Hôpital Tenon, Paris, France; and Centre de Recherche du CHU Ste-Justine, Département de Pédiatrie, Université de Montréal, Montréal, Québec, Canada

Correspondence: Dr. Elie Haddad, Centre de Recherche du CHU Suite-Justine, Département de Pédiatrie, Université de Montréal, 3175 Cote Sainte-Catherine, Montréal, QC H3T1C5, Canada. Phone: 514-345-4713; Fax: 514-345-4897; E-mail: elie.haddad{at}umontreal.ca

Received for publication December 12, 2006. Accepted for publication June 26, 2007.

Idiopathic nephrotic syndrome is characterized by glomerular proteinuria in the absence of infiltrating cells or immunoglobulin deposits. Although it is suspected that T cells secrete a circulating factor that leads to proteinuria by altering the permeability of the glomerular filtration barrier, the precise etiology of this syndrome is unknown. Because an animal model that mimics human idiopathic nephrotic syndrome does not exist, we developed a humanized mouse model of the disease by injecting CD34⁺ stem cells or CD34^– peripheral blood mononuclear cells from afflicted patients into immunocompromised mice. Even though both CD34⁺ and CD34^– cells induced the engraftment of human CD45⁺ leukocytes in mice, only the injection of CD34⁺ stem cells induced albuminuria. Ultrastructural analysis of glomeruli from the resulting proteinuric mice revealed effacement of podocyte foot processes, similar to the pathology observed in the human disease. Therefore, our data suggest that the cells responsible for the pathogenesis of idiopathic nephrotic syndrome are more likely to be immature differentiating cells rather than mature peripheral T cells.

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Copyright © 2008 by the American Society of Nephrology. Online ISSN: 1533-3450 Print ISSN: 1046-6673