Dilated cardiomyopathy: concepts derived from gene deficient and transgenic animal models.
Genetic forms of human dilated cardiomyopathy (DCM) are briefly discussed, and a variety of animal models of genetic DCM are presented, some of which are caused by the gene mutations that also cause DCM in humans. The forms of DCM related to mutations or deletion of genes coding for extrasarcomeric or intrasarcomeric proteins, as well as to overexpression or knockout of genes in the beta-adrenergic signaling pathway, are included. Finally, novel approaches to treatment in experimental animal models are discussed, including double transgenesis and newer recombination methods, as well as in vivo somatic gene transfer which, based on initial experiments in animals, seems likely to find eventual application in human cardiac failure.
Ross
John
J
Department of Medicine, Institute of Molecular Medicine, University of California, San Diego 92093-0613, USA. jross@ucsd.edu
D000818:Animals; D030801:Animals, Genetically Modified; D002311:Cardiomyopathy, Dilated / Q000209:etiology / Q000235:genetics* / Q000628:therapy; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D009154:Mutation