Article
Progressive atrioventricular conduction defects and heart failure in mice expressing a mutant Csx/Nkx2.5 homeoprotein.
The Journal of clinical investigation - 1 Jul 2001
Kasahara H, Wakimoto H, Liu M, Maguire C T, Converso K L, Shioi T, Huang W Y, Manning W J, Paul D, Lawitts J, Berul C I, Izumo S
Abstract excerpt
A DNA nonbinding mutant of the NK2 class homeoprotein Nkx2.5 dominantly inhibits cardiogenesis in Xenopus embryos, causing a small heart to develop or blocking heart formation entirely. Recently, ten heterozygous CSX/NKX2.5 homeoprotein mutations were identified in patients with congenital atrioventricular (AV) conduction defects. All four missense mutations identified in the human homeodomain led to markedly...
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