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Kids Kidney Research

Kids Kidney Research

A mechanistic link between plasma factors and podocyte dysfunction in Focal Segmental Glomerulosclerosis

Nephrotic syndrome is a disease of kidney filtration, whereby huge amounts of protein are lost in the urine due to a defect in the primary filtering cell of the kidney, the podocyte. In affected children this leads to swelling (oedema), susceptibility to infections, clotting risk and eventually kidney failure. The treatment is often poorly effective, and involves long courses of toxic immunosuppression A particularly difficult complication is that the disease can rapidly recur in a transplanted kidney. This has led to the theory that the disease is caused by abnormalities in circulating plasma.

The researchers have developed unique resources in the laboratory whereby they can test what elements of human plasma cause damage in nephrotic syndrome. Their's is the only laboratory in the world to have developed human podocytes to grow and study, which provides the ideal model system. This study proposes to study elements in human plasma called proteases, and work out whether they cause damage to the podocyte likely to reflect the damage seen in nephrotic syndrome. If successful, it will identify specific targets in the podocyte and in plasma, which could be used to alleviate or cure nephrotic syndrome.

 

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