Stress-induced dilated cardiomyopathy in a knock-in mouse model mimicking human titin-based disease.

No abstract is available for this paper.

• Publication year

  2009

• Venue

  Journal of Molecular and Cellular Cardiology

• Publication date

  2009-09-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1016/j.yjmcc.2009.04.014PMID 19406126PMCID PMC2764556
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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  1997cited by this paper
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  1995cited by this paper
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  1995cited by this paper
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  1994cited by this paper
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  1990cited by this paper
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