Loss of enigma homolog protein results in dilated cardiomyopathy.

ABSTRACT:

Rationale

The Z-line, alternatively termed the Z-band or Z-disc, is a highly ordered structure at the border between 2 sarcomeres. Enigma subfamily proteins (Enigma, Enigma homolog protein, and Cypher) of the PDZ-LIM domain protein family are Z-line proteins. Among the Enigma subfamily, Cypher has been demonstrated to play a pivotal role in the structure and function of striated muscle, whereas the role of Enigma homolog protein (ENH) in muscle remains largely unknown.

Objective

We studied the role of Enigma homolog protein in the heart using global and cardiac-specific ENH knockout mouse models.

Methods and results

We identified new exons and splice isoforms for ENH in the mouse heart. Impaired cardiac contraction and dilated cardiomyopathy were observed in ENH null

SUBMITTER: Cheng H 

PROVIDER: S-EPMC3684396 | biostudies-literature | 2010 Aug

REPOSITORIES: biostudies-literature