C4-2 cells that stably express PKD1-GFP was previously described

lic Function LV Ejection Fraction Vcfc, circumference/sec Sa Spw Left Ventricular Diastolic Function IVRT Ea Epw E/Ea Data represent the means 6 SE. Statistical significance was determined by unpaired, two-tailed t-test: P,0.05: P,0.01. Abbreviations: LA, left atrium; LV, left ventricle; LV EDD, left ventricular end diastolic diameter; Vcfc, velocity of shortening of circumferential fibers; Sa, Spw: maximal systolic velocity of the mitral annulus and posterior wall; IVRT: isovolumic relaxation time; Ea and Epw: maximal diastolic velocity of the mitral annulus and posterior wall; E, maximal velocity of LV inflow. Staining with Picrosirius Red showed no significant differences in non-vascular collagen staining in the myocardium of male Fabry KO mice compared to WT mice and no predominant area of fibrosis in Fabry KO mice. Systolic Blood Pressure, Heart Rate, ECG and Cardiac Weight Measurements: Effects of ERT The measurements of RR intervals with surface ECG recordings showed identical RR intervals and the standard deviation of the RR intervals, and the frequency of premature atrial contractions for Fabry KO mice compared to Fabry KO mice treated with ” 3 mg/kg intravenous ERT 3 weeks before. There were no significant differences in PQ, QRS, or corrected QT intervals. ERT had no effect 3 weeks after injection on heart weight for Fabry KO mice, when normalized to body weight or tibial length. There was no effect 3 weeks after injection on heart weight for WT mice, when normalized to body weight or tibial length. LV Molecular Alterations in Fabry KO Mice We examined myocardial alterations that could be associated with cardiac remodeling and dysfunction by analyzing mRNA expression for atrial natriuretic factor brain natriuretic peptide, plasminogen activator inhibitor-1; connective tissue ” growth factor, thrombospondin 1, and thrombospondin 2. As”
13679412” shown in Left Ventricle and (S)-(-)-Blebbistatin supplier Aortic Structural Alterations with ERT The echocardiography results for 67 month-old Fabry KO mice are summarized in 4 Cardiomyopathy in Fabry Mouse Model age-matched controls according to a trend toward decrease in LV diameter after treatment without change in wall thickness. There was a significant decrease in the aortic diameter during diastole for Fabry KO mice treated with ERT compared to the age-matched KO controls. Left Ventricular Functional Alterations in Fabry KO Mice with ERT The heart rates obtained in 67 week old mice undergoing echocardiography with isoflurane anesthesia were the same for untreated Fabry KO mice compared to age-matched Fabry KO mice that had received ERT as a single IV injection 3 weeks before evaluation. LV systolic function, as assessed by echocardiography was similar in ERT-treated Fabry KO mice as compared to agematched untreated KO mice. LV Molecular Alterations in Fabry KO and WT Mice Treated with ERT As shown in mRNA normalized to GAPDH levels in ERT-treated WT mice, but there were not any effects of ERT on any of the mRNA levels examined in this series compared to WT concurrent control male mice. Discussion In the present study, we define the cardiac phenotype in male Fabry KO mice. We found that these mice have bradycardia and low systemic blood pressures compared to the control WT strain, as well as mild hypertrophic cardiomyopathy on echocardiography and gravimetry. The heart rates and systolic blood pressures reported herein for the WT mice are very similar to previous reports. Thus KO Fabry mice displayed a mild hypertrophic cardi