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Publication Detail
Loss of Prox1 in striated muscle causes slow to fast skeletal muscle fiber conversion and dilated cardiomyopathy.
  • Publication Type:
    Journal article
  • Publication Sub Type:
    Journal Article
  • Authors:
    Petchey LK, Risebro CA, Vieira JM, Roberts T, Bryson JB, Greensmith L, Lythgoe MF, Riley PR
  • Publication date:
  • Pagination:
    9515, 9520
  • Journal:
    Proc Natl Acad Sci U S A
  • Volume:
  • Issue:
  • Status:
  • Country:
    United States
  • PII:
  • Language:
  • Keywords:
    Animals, Cardiomyopathy, Dilated, Cell Differentiation, Chromatin Immunoprecipitation, DNA Primers, Fluorescent Antibody Technique, Gene Expression Regulation, Developmental, Homeodomain Proteins, Mice, Mice, Knockout, Microarray Analysis, Muscle, Striated, Myocardium, Myosin Light Chains, Real-Time Polymerase Chain Reaction, Troponin, Troponin I, Tumor Suppressor Proteins
Correct regulation of troponin and myosin contractile protein gene isoforms is a critical determinant of cardiac and skeletal striated muscle development and function, with misexpression frequently associated with impaired contractility or disease. Here we reveal a novel requirement for Prospero-related homeobox factor 1 (Prox1) during mouse heart development in the direct transcriptional repression of the fast-twitch skeletal muscle genes troponin T3, troponin I2, and myosin light chain 1. A proportion of cardiac-specific Prox1 knockout mice survive beyond birth with hearts characterized by marked overexpression of fast-twitch genes and postnatal development of a fatal dilated cardiomyopathy. Through conditional knockout of Prox1 from skeletal muscle, we demonstrate a conserved requirement for Prox1 in the repression of troponin T3, troponin I2, and myosin light chain 1 between cardiac and slow-twitch skeletal muscle and establish Prox1 ablation as sufficient to cause a switch from a slow- to fast-twitch muscle phenotype. Our study identifies conserved roles for Prox1 between cardiac and skeletal muscle, specifically implicated in slow-twitch fiber-type specification, function, and cardiomyopathic disease.
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Department of Neuromuscular Diseases
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Experimental & Translational Medicine
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