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Background: Myosin binding protein-H like (MyBP-HL) is a recently identified atrial myofilament protein encoded by the gene MYBPHL. The Arg255Stop mutant has been linked to atrial enlargement, atrial fibrillation, and dilated cardiomyopathy. Deletion of MYBPHL in mice recapitulates this human phenotype. MyBP-HL is composed of two Immunoglobulin dom...
Cardiomyopathy and arrhythmia are diseases with significant genetic linkage. Cardiac myosin binding protein-C (cMyBP-C) is a myofilament associated protein with a C-terminal composed of repeating immunoglobulin (Ig) and fibronectin III (FnIII) domains that bind to the myosin thick filaments and titin. Truncating mutations in the C-terminal of MyBP-...
Myosin binding protein-H like (MyBP-HL) has recently been identified as a novel component of myofilaments in the cardiac atria. Loss of MyBP-HL is associated with dilated cardiomyopathy and arrhythmia in humans and mice. MyBP-HL is highly homologous to cardiac myosin binding protein-C (cMyBP-C), which has been studied in the context of genetic card...