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Nephrol Dial Transplant (2002) 17: 1359-1362
© 2002 European Renal Association-European Dialysis and Transplant Association

Editorial Comments

Animal models of Alport syndrome

Clifford E. Kashtan

Department of Pediatrics, University of Minnesota Medical School, Minneapolis, Minnesota, USA

The first 10% of the full text of this article appears below.

Introduction

The last decade of the twentieth century was a very productive period for the study of Alport syndrome. Alport syndrome was shown to result from mutations in certain members of the type IV collagen family of proteins, the {alpha}3(IV), {alpha}4(IV), and {alpha}5(IV) chains. Several hundred different mutations in the COL4A5 gene, which encodes the {alpha}5(IV) chain, were described in patients with X-linked Alport syndrome [1]. A few dozen mutations were found in the COL4A3 and COL4A4 genes, which respectively encode the {alpha}3(IV) and {alpha}4(IV) chains, in patients with autosomal recessive Alport syndrome [2,3]. Autosomal dominant Alport syndrome, due to heterozygous mutations in COL4A3 or COL4A4, was established as an entity, and distinguished from Fechtner and Epstein syndromes, which arise from mutations in a non-collagen locus, MYH9 [4–7]. Investigators established that mutations in the {alpha}3(IV), {alpha}4(IV), or {alpha}. . . [Full Text of this Article]

X-linked Alport syndrome (XLAS)

Autosomal recessive Alport syndrome (ARAS)

Autosomal dominant Alport syndrome (ADAS)

Reseach applications of animal models of Alport syndrome

Note added in proof


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