Frémeaux-Bacchi V, Arzouk N, Ferlicot S, Charpentier B, Snanoudj R, Dürrbach A
Immunology Department, Hôpital Européen Georges Pompidou, Paris, France.
Am J Transplant. 2007 Aug;7(8):2047-51. doi: 10.1111/j.1600-6143.2007.01888.x.
Mutations in the gene of the membrane cofactor protein (MCP/CD46), a complement regulatory protein, were recently described as a cause of hemolytic uremic syndrome (HUS). MCP is a transmembrane glycoprotein expressed in kidneys; therefore, the transplantation of a normal kidney should not be complicated by HUS recurrence. However, we report the case of a 32-year-old woman with an MCP mutation who developed a recurrence of HUS after renal transplantation. We found that she had vascular microchimerism of endothelial cells. We suggest that recurrence may be favored by vascular microchimerism, in which the mutated protein is produced in the in the kidney graft by endothelial cells originating from recipient.
膜辅因子蛋白（MCP/CD46）是一种补体调节蛋白，该基因的突变最近被认为是溶血尿毒综合征（HUS）的病因。MCP是一种在肾脏中表达的跨膜糖蛋白；因此，移植正常肾脏不应因HUS复发而变得复杂。然而，我们报告了一例32岁患有MCP突变的女性，她在肾移植后发生了HUS复发。我们发现她存在内皮细胞的血管微嵌合体。我们认为，血管微嵌合体可能有利于复发，在这种情况下，移植肾中源自受者的内皮细胞会产生突变蛋白。
