Table 2.

Summary of mouse models generated to determine the role of mechanical components of ICD (fascia adherens junction and desmosomes) in postnatal myocardium and cardiac disease

ICD-associated junctional complexes	ICD component	Mouse models and cardiac phenotypes	Reference(s)
Fascia adherens junction	N-cadherin	CS-overexpression: DCM (mild), cardiac calcification, thrombosis	Li et al. 2006
		CS-I-KO: DCM (modest), ventricular arrhythmias, sudden cardiac death	Li et al. 2006, Li et al. 2008
	α-E-catenin	CS-KO: progressive DCM, RV defects, Predisposed to MI-induced ventricular rupture	Sheikh et al. 2006
		CS-I-KO: predisposed to MI-induced ventricular rupture	Van den Borne et al. 2008
	β-Catenin	CS-KO: no basal phenotype but blunted response to hypertrophy following TAC	Chen et al. 2006
		CS-I-KO: no basal phenotype due to compensation by plakoglobin	Zhou et al. 2007
	Vinculin	Heterozygous KO: predisposed to postnatal sudden death after TAC	Zemljic-Harpf et al. 2004
		CS-KO: postnatal sudden death via ventricular tachycardia, DCM	Zemljic-Harpf et al. 2007
	mXinα	Conventional KO: postnatal cardiac hypertrophy, cardiomyopathy, and conduction defects	Gustafson-Wagner et al. 2007
	LIMP-2	Conventional KO: viable but blunted response to hypertrophy after TAC, DCM after angiotensin II treatment	Schroen et al. 2007
	CAR a	CS-KO: viable but exhibit postnatal AV conduction defects and DCM	Lim et al. 2008, Lisewski et al. 2008
Desmosomes	Plakoglobin a	Heterozygous KO: viable but displayed postnatal cardiac abnormalities associated with ARVC, including exacerbation in ventricular arrhythmias after exercise	Kirchhof et al. 2006
	Desmoplakin	CS (α-MHC-Cre)-KO: lethality at E10–E12 with defects in chamber specification and myocyte organization; embryonic survivors died at 2 weeks after birth	Garcia-Gras et al. 2006
		CS (α-MHC-Cre) heterozygous KO: features associated with ARVC	Garcia-Gras et al. 2006
		CS-R283H mutation overexpression: features associated with ARVC	Yang et al. 2006

KO indicates knockout; CS, cardiac-specific; CS-I, cardiac-specific-inducible; MI, myocardial infarction; TAC, transverse aortic constriction.

^aIt should be noted that CAR is also present within gap junctions and that plakoglobin is also found at the fascia adherens junction.