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1. Nkx2-5 pathways and congenital heart disease: loss of ventricular myocyte lineage specification leads to progressive cardiomyopathy and complete heart block

Author

Pashmforoush, Mohammad, Jonathan T. Lu, Hanying Chen, Amand, Tara St., Kondo, Richard, Pradervand, Sylvain, Evans, Sylvia M., Clark, Bob, Feramisco, James R., Silberbach, Michael, Shou, Weinian, Giles, Wayne, Siew Yen Ho, Benson, D. Woodrow, and Kenneth R. Chien

Subjects
Gene mutations -- Research, Cardiomyopathy -- Development and progression, Heart diseases -- Development and progression, Biological sciences
Abstract

A study addressing the problem of human mutations in Nkx2-5 leading to progressive cardiomyopathy and conduction defects via unknown mechanisms, by generating mice with a ventricular muscle cell-restricted knockout of Nkx2-5 is presented. The results show that loss of ventricular muscle cell lineage specification into trabecular and conduction system myocytes is a new mechanistic pathway for cardiomyopathy and conduction defects in congenital heart disease.

Published
2004

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