Objective To establish the heart-specific expression Calponin 1 transgenic mice and investigate its effects on the development of heart and cardiomyopathy. Method The transgenic vector was constructed by inserting the human Calponin 1 gene into the down-stream of α-MHC promoter. The transgenic mice were generated by the method of microinjection. The genotype of transgenic lines was identified by PCR，and the expression levels of the Calponin 1 gene were detected by Western Blot. The pathologic changes of the heart structure and function were analyzed by echocardiography，hematoxylin and eosin( HE) stain and Masson stain. Result The expression of Calponin 1 was detected in the heart of different age wide-type mice by Western Blot，but was down-regulated in the mice with DCM. Compared with the wild type mice，transgenic mice showed significant heart remodeling，left ventricular systolic diameter increased by28% (P < 0. 01，n = 12) ，left ventricular diastolic diameter elevated by 16. 2% (P < 0. 01，n = 12) ，left ventricular posterior wall during systolic decreased by 15. 7% (P < 0. 01，n = 12) ，and left ventricular posterior wall during diastole reduced by 21% (P < 0. 01，n = 12) . Transgenic mice，versed wild type，showed weakened heart function，ejection fraction diminished by 11. 5% (P < 0. 01，n = 12) and fraction shortening reduced by 14. 6% (P < 0. 05，n = 12) .The heart of the transgenic mice exhibited an enlarged ventricular chamber when compared with that of the wild type with HE stain and Masson stain. Conclusion Over-expression of Calponin 1 in the heart of the transgenic mice caused a phenotype similar with dilated cardiomyopathy. It suggested that Calponin 1 might be one of the modifier genes which involved in the pathogenesis of cardiomyopathy.