A transgenic rabbit model for human hypertrophic cardiomyopathy.

No abstract is available for this paper.

• Publication year

  1999

• Venue

  Journal of Clinical Investigation

• Publication date

  1999-12-15

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1172/JCI7956PMID 10606622PMCID PMC409884
• 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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  1999influential reference
• Identification of a contractile deficit in adult cardiac myocytes expressing hypertrophic cardiomyopathy-associated mutant troponin T proteins.

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  1998cited by this paper
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  1998cited by this paper
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