This transgene contains the promoter and regulatory sequences of the human actin, alpha 1, skeletal muscle (ACTA1) gene driving expression of a full-length utrophin cDNA with the SV40 large T antigen polyadenylation signal. Western blot analysis of extracts of tibialis anterior (TA), extensor digitalis longus (EDL) and diaphragm muscle using a utrophin-specific monoclonal antibody (MANCHO3) demonstrates utrophin expression in these muscles of transgenic Dmdmdx/Dmdmdx mice at similar or lower levels than the normally high level expression from the endogenous gene in lung and kidney. Immunohistoluminescent analysis reveals relocalization of utrophin to the sarcolemma of transgenic Dmdmdx/Dmdmdx tibialis anterior muscle. (J:51364)
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cx: complex: > 1 genome feature ot: other: hemizygous, indeterminate,... (F): Female
(M): Male
N: normal phenotype
(#): related diseases count