Review
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World J Nephrol. Nov 6, 2014; 3(4): 230-236
Published online Nov 6, 2014. doi: 10.5527/wjn.v3.i4.230
Searching for a treatment for Alport syndrome using mouse models
Kan Katayama, Shinsuke Nomura, Karl Tryggvason, Masaaki Ito
Kan Katayama, Shinsuke Nomura, Masaaki Ito, Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Tsu, Mie 514-8507, Japan
Kan Katayama, Karl Tryggvason, Division of Matrix Biology, Department of Medical Biochemistry and Biophysics, Karolinska Institute, Stockholm S-17177, Sweden
Author contributions: Katayama K, Nomura S, Tryggvason K and Ito M contributed to writing this paper.
Correspondence to: Kan Katayama, MD, PhD, Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie 514-8507, Japan. katayamk@clin.medic.mie-u.ac.jp
Telephone: +81-59-2321111
Received: June 16, 2014
Revised: July 15, 2014
Accepted: September 16, 2014
Published online: November 6, 2014
Core Tip

Core tip: There is currently no curative treatment for Alport syndrome, a progressive hereditary nephritis. However, many drugs have been demonstrated to slow the progression of renal injury in Alport mouse models. Alport mice treated with vasopeptidase inhibitors or angiotensin-converting enzyme inhibitors showed a more than two-fold longer survival than untreated Alport mice. A human clinical trial of an angiotensin-converting enzyme inhibitor is currently in progress. Genetic approaches have been used to elucidate the pathogenesis of this progressive renal disease. Stem cell therapies were also attempted, with some beneficial effects; however, they need to be improved before being tested in clinical trials.