A novel mutant gene inhibiting the progression of autoimmune glomerulonephritis

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Crescentic glomerulonephritis (CreGN) is a representative outcome of autoimmune glomerulopathy, morphologically characteristics of extracapillary proliferative lesions in renal Bowman's space. Its rapid progression for irreversible renal failureis is of clinical importance. We previously had reported the establishment of murine disease-model (named EOD strain), of which the mice die of CreGN by 3 month-old. Recently, among these EOD mice, we found a spontaneous mutant strain of mice that exhibited much longer life-span and improved CreGN. Comparative pathological study using wild-type disease and mutant healthy mice demonstrated that a defect in platelet function was responsible for the improvement of CreGN. Furthermore, genetic analysis successfully identified the gene of mutation, which turned out to code a novel protein. Protein analysis showed that this mutation caused a loss of expression of this protein. The present study put forth an idea for therapeutic strategy against CreGN, that targets that molecular or a platelet function.