Utrophin expression during human fetal development |
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Authors: | C. Rigoletto A. Prelle P. Ciscato M. Moggio G. Comi F. Fortunato G. Scarlato |
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Affiliation: | Istituto di Clinica Neurologica, Università di Milano, Centro Dino Ferrari, Milan, Italy |
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Abstract: | Utrophin, a protein encoded by chromosome 6 is highly homologous to the cysteine-rich domain and most of the C-terminal domain of dystrophin. In order to clarify its functional role we analyzed its expression during human fetal development. We carried out immunohistochemical analysis on muscle from normal human fetuses at different ages of gestation using an antibody directed against a specific COOH-terminal sequence of the protein. In addition, we stained serial sections with antibodies against dystrophin and alpha-bungarotoxin FITC-BTX. Our findings show that, at week 9 of gestation, utrophin is diffusely expressed in the cytoplasm. From week 12 to 22 the immunostaining is still cytoplasmic, though the reaction intensity progressively decreases. Moreover we observed a strong reaction in fetal nerve at week 18 and 22. There was no correlation between utrophin expression and progressive dystrophin membrane localization. |
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Keywords: | dystrophin utrophin human fetal development |
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