Open AccessDesensitization of Myofilaments to Ca 2+ as a Therapeutic Target for Hypertrophic Cardiomyopathy With Mutations in Thin Filament Proteins
Author(s) -
Marco Stephan LofranoAlves,
Fernando Augusto Lavezzo Dias,
Robert D. Gaffin,
Jillian N. Simon,
Eric M. Montminy,
Brandon J. Biesiadecki,
Aaron C. Hinken,
Chad M. Warren,
Megan S. Utter,
Robert T. Davis,
Sakthivel Sadayappan,
Jeffrey Robbins,
David F. Wieczorek,
R. John Solaro,
Beata M. Wolska
Publication year - 2014
Publication title -
circulation cardiovascular genetics
Language(s) - English
Resource type - Journals
eISSN - 1942-325X
pISSN - 1942-3268
DOI - 10.1161/circgenetics.113.000324
Subject(s) - myofilament , phospholamban , medicine , endocrinology , sarcomere , troponin i , muscle hypertrophy , heart failure , cardiomyopathy , hypertrophic cardiomyopathy , tropomyosin , genetically modified mouse , cardiology , biology , chemistry , myocyte , transgene , myosin , microbiology and biotechnology , biochemistry , myocardial infarction , gene
Hypertrophic cardiomyopathy (HCM) is a common genetic disorder caused mainly by mutations in sarcomeric proteins and is characterized by maladaptive myocardial hypertrophy, diastolic heart failure, increased myofilament Ca(2+) sensitivity, and high susceptibility to sudden death. We tested the following hypothesis: correction of the increased myofilament sensitivity can delay or prevent the development of the HCM phenotype.
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