Berger in 1968.1 Histopathologically,

IgA nephropathy is characterized by expansion of the glomerular mesangial matrix with mesangial cell proliferation. Glomeruli typically contain generalized-diffuse granular mesangial Veliparib deposits of IgA (mainly IgA1), IgG and C3. Clinically, patients with IgA nephropathy showed microscopic and/or macroscopic haematuria and/or proteinuria. Advanced patients progress to renal hypertension and end-stage kidney disease (ESKD). Approximately 30–40% of patients with IgA nephropathy develop hypertension and progress to ESKD. Recognizing those patients likely to progress to ESKD and identifying suitable therapeutic targets are major goals for nephrologists. Central to achieving these goals is the development of suitable animal models to provide a detailed understanding of the underlying pathogenesis of IgA nephropathy. Because pathogenesis and radical treatment for IgA nephropathy are still not established, it is necessary to study them using animal models.2,3 Several investigators, including Rifai et al.4 and Emancipator et al.,5 reported experimental animal models for IgA nephropathy.

In 1985, Imai et al.6 first reported that the ddY strain of mouse can serve as a spontaneous animal model for human IgA nephropathy. These mice show mild proteinuria without haematuria and mesangioproliferative glomerulonephritis with severe glomerular find more IgA deposits in association with an increase in serum IgA level. Marked deposition of IgA and C3 in the glomerular mesangial areas in association with an increase in the levels of macromolecular IgA appears in sera of these mice with aging. Electron-dense deposits are observed in the

glomerular mesangial areas by electron microscopy. These findings appear at more than 40 weeks of age. It was found that ddY mice derived from non-inbred dd-stock selleck kinase inhibitor mice brought from Germany before 1920 and then raised in Japan developed spontaneously IgA-dominant deposition in the glomerular mesangium.6 Muso et al.7 reported that dimeric and polymeric IgA can be eluted from diseased glomeruli of aged ddY mice. However, the incidence of IgA nephropathy in ddY mice is highly variable. Miyawaki et al.8 succeeded in generating an IgA nephropathy mouse with a high incidence and early onset of glomerular IgA deposition. The selected ddY line (high serum IgA ddY (HIGA) mice) showed only mild proteinuria (100–300 mg/dL) without haematuria. It appears that immunological aberrations in ddY mice resemble those in human IgA nephropathy although these mice did not show microscopic haematuria and severe glomerular injuries. These findings from ddY mice appear to be useful in studying the pathogenesis and treatment for patients with IgA nephropathy.