Essential roles of an intercalated disc protein, mXinbeta, in postnatal heart growth and survival.

ABSTRACT:

Rationale

The Xin repeat-containing proteins mXinalpha and mXinbeta localize to the intercalated disc of mouse heart and are implicated in cardiac development and function. The mXinalpha directly interacts with beta-catenin, p120-catenin, and actin filaments. Ablation of mXinalpha results in adult late-onset cardiomyopathy with conduction defects. An upregulation of the mXinbeta in mXinalpha-deficient hearts suggests a partial compensation.

Objective

The essential roles of mXinbeta in cardiac development and intercalated disc maturation were investigated.

Methods and results

Ablation of mXinbeta led to abnormal heart shape, ventricular septal defects, severe growth retardation, and postnatal lethality with no upregulation of the mXinalpha. Postnatal upregulation of mX

SUBMITTER: Wang Q 

PROVIDER: S-EPMC2872156 | biostudies-literature | 2010 May

REPOSITORIES: biostudies-literature