Disruption of the Sp1-related transcription factor HF-1b leads to alterations in the cardiac conduction system that result in ventricular arrhythmias and sudden death. What does this tell us about how ventricular cardiomyocytes differentiate into the specialized cells that form the heart's conductance system?
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References
Geisterfer-Lowrance, A.A. et al. A molecular basis for familial hypertrophic cardiomyopathy: a beta cardiac myosin heavy chain gene missense mutation. Cell 62, 999–1006 (1990).
Wang, Z. et al. Functional effects of mutations in KvLQT1 that cause long QT syndrome. J. Cardiovasc. Electrophysiol. 10, 817–826 (1999).
Ackerman, M.J. The long QT syndrome: ion channel diseases of the heart. Mayo Clin. Proc. 73, 250–269 (1998).
Schott, J.J. et al. Congenital heart disease caused by mutations in the transcription factor NKX2-5. Science 281, 108–111 (1998).
Basson, C.T. et al. Mutations in human TBX5 cause limb and cardiac malformation in Holt-Oram syndrome. Nature Genet. 15, 30–35 (1997).
Nguyen-Tran, V.T.B. et al. A novel genetic pathway for sudden cardiac death via defects in the transition between ventricular and conduction system cell lineages. Cell, (in the press).
Supp, D.M., Witte, D.P., Branford, W.W., Smith, E.P. & Potter, S.S. Sp4, a member of the Sp1-family of zinc finger transcription factors, is required for normal murine growth, viability, and male fertility. Dev. Biol. 176, 284–299 (1996).
Takebayashi-Suzuki, K., Yanagisawa, M., Gourdie, R.G., Kanzawa, N. & Mikawa, T. In vivo induction of cardiac Purkinje fiber differentiation by co-expression of preproendothelin-1 and endothelin converting enzyme-1. Development 127, 3523–3532 (2000).
Kupershmidt, S. et al. Replacement by homologous recombination of the minK gene with lacZ reveals restriction of minK expression to the mouse cardiac conduction system. Circ. Res. 84, 146–152 (1999).
Sussman, M.A. et al. Myofibril degeneration caused by tropomodulin overexpression leads to dilated cardiomyopathy in juvenile mice. J. Clin. Invest. 101, 51–61 (1998).
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Robbins, J., Dorn, G. Listening for hoof beats in heart beats. Nat Med 6, 968–970 (2000). https://doi.org/10.1038/79652
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DOI: https://doi.org/10.1038/79652
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