A mouse model of conduction system patterning abnormalities in heterotaxy syndrome.

ABSTRACT: Duplication or absence of parts of the specialized cardiac conduction system in patients with heterotaxy syndrome causes significant clinical disease, but the mechanistic basis by which embryonic disruption of left-right patterning alters conduction system patterning in these patients is not well understood. We sought to determine whether a mouse model of X-linked human heterotaxy recapitulates conduction system abnormalities identified in patients with heterotaxy. Cardiac structure and conduction system patterning were evaluated in Zic3 null embryos from e9.5 to e16.5 using genetic and molecular methods. Severe structural abnormalities involving atrial, ventricular, and conotruncal development were associated with a spectrum of disorganized and ambiguous arrangements throughout the conduc

SUBMITTER: Czosek RJ 

PROVIDER: S-EPMC2940945 | biostudies-literature | 2010 Oct

REPOSITORIES: biostudies-literature