PUBLICATION

Distinct troponin C isoform requirements in cardiac and skeletal muscle

Authors
Sogah, V.M., Serluca, F.C., Fishman, M.C., Yelon, D.L., MacRae, C.A., and Mably, J.D.
ID
ZDB-PUB-101011-50
Date
2010
Source
Developmental dynamics : an official publication of the American Association of Anatomists   239(11): 3115-3123 (Journal)
Registered Authors
Fishman, Mark C., Mably, John, MacRae, Calum A., Serluca, Fabrizio, Yelon, Deborah
Keywords
zebrafish, troponin C, contractility, cardiovascular, heart
MeSH Terms
  • Animals
  • In Situ Hybridization
  • Microscopy, Electron, Transmission
  • Muscle, Skeletal/metabolism*
  • Muscle, Skeletal/ultrastructure
  • Myocardium/metabolism*
  • Myocardium/ultrastructure
  • Protein Isoforms/genetics
  • Protein Isoforms/metabolism*
  • Troponin C/genetics
  • Troponin C/metabolism*
  • Zebrafish
PubMed
20925115 Full text @ Dev. Dyn.
Abstract
The zebrafish mutant silent partner is characterized by a dysmorphic, non-contractile ventricle resulting in an inability to generate normal blood flow. We have identified the genetic lesion in the zebrafish homolog of the slow twitch skeletal/cardiac troponin C gene. Although human troponin C1 (TNNC1) is expressed in both cardiac and skeletal muscle, duplication of this gene in zebrafish has resulted in tissue-specific partitioning of troponin C expression and function. Mutation of the zebrafish paralog tnnc1a, which is expressed predominantly in the heart, results in a loss of contractility and myofibrillar organization within ventricular cardiomyocytes, while skeletal muscle remains functional and intact. We further show that defective contractility in the developing heart results in abnormal atrial and ventricular chamber morphology. Together, our results suggest that tnnc1a is required both for the function and structural integrity of the contractile machinery in cardiomyocytes, helping to clarify potential mechanisms of troponin C-mediated cardiomyopathy.
Genes / Markers
Figures
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Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping