Cardiac Myosin Binding Protein C Gene Is Specifically Expressed in Heart During Murine and Human Development

From the Laboratoire d'Histo-Embryologie et de Cytogénétique (F.F.), Faculté Cochin Port-Royal, Paris, France; Service d'Histo-Embryologie et de Cytogénétique (A.-L.D.), Hôpital Necker, Paris, France; the Généthon (F.F., J.S.B.), URA1922 C.N.R.S., Evry, France; the INSERM Unit 153 and the IFR de Physiopathologie et Génétique Cardiovasculaire (M.Y.F., K.S., L.C.), Groupe Hospitalier Pitié-Salpétrière, Paris, France.

Correspondence to Dr Lucie Carrier, INSERM UR153, Institut de Myologie, Rue du Mur des Fermiers Généraux, Groupe Hospitalier Pitié-Salpétrière, 47 boulevard de l'Hôpital, 75651 Paris cédex 13, France. E-mail lcarrier{at}myologie.infobiogen.fr

Abstract—Cardiac myosin binding protein C (MyBP-C) is a substantial component of the sarcomere, with both structural and regulatory roles. The gene encoding cardiac MyBP-C in humans is located on chromosome 11p11.2, and mutations that are most predicted to produce truncated proteins have been identified in this gene in unrelated families with familial hypertrophic cardiomyopathy (FHC). To understand better the pathophysiology of FHC and with a view to the development of animal models for this disease, we have investigated by in situ hybridization the pattern of expression of the cardiac MyBP-C gene during human and mouse development using species-specific oligonucleotide probes. From 4 weeks of human development, a strong labeling of cardiac MyBP-C mRNAs was unambiguously detected in all heart compartments, and no signal could be visualized in somites. In murine embryos, from embryonic day 9.5 until birth, a strong signal was detected exclusively in the heart. Our results showed that during both human and murine development, in contrast to chicken development, the cardiac MyBP-C gene is abundantly and specifically expressed in the heart.

Key Words: myosin binding protein C • cardiac muscle • mouse development • human development • expression

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