期刊
JOURNAL OF THE AMERICAN HEART ASSOCIATION
卷 4, 期 5, 页码 -出版社
WILEY-BLACKWELL
DOI: 10.1161/JAHA.115.001950
关键词
Cypher; embryonic lethality; Enigma homolog protein; Z-line
资金
- National Institute of Arthritis and Musculoskeletal and Skin [R01AR059334]
- National Heart, Lung, and Blood Institute
- National Institutes of Health [5T32HL007444-27, K99HL107744-01]
- National Key Basic Research Program of China [2013CB531200]
- National Science Foundation of China [31370823, 91439130]
Background-The striated muscle Z-line, a multiprotein complex at the boundary between sarcomeres, plays an integral role in maintaining striated muscle structure and function. Multiple Z-line-associated proteins have been identified and shown to play an increasingly important role in the pathogenesis of human cardiomyopathy. Cypher and its close homologue, Enigma homolog protein (ENH), are 2 Z-line proteins previously shown to be individually essential for maintenance of postnatal cardiac function and stability of the Z-line during muscle contraction, but dispensable for cardiac myofibrillogenesis and development. Methods and Results-The current studies were designed to test whether Cypher and ENH play redundant roles during embryonic development. Here, we demonstrated that mice lacking both ENH and Cypher exhibited embryonic lethality and growth retardation. Lethality in double knockout embryos was associated with cardiac dilation and abnormal Z-line structure. In addition, when ENH was ablated in conjunction with selective ablation of either Cypher short isoforms (CypherS), or Cypher long isoforms (CypherL), only the latter resulted in embryonic lethality. Conclusions-Cypher and ENH redundantly play an essential role in sustaining Z-line structure from the earliest stages of cardiac function, and are redundantly required to maintain normal embryonic heart function and embryonic viability.
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