To determine if decreased C/EBPb binding by C/EBPc could lead to the reduced IL-6 expression, MLE 12 cells were transfected with C/EBPb plasmid in the presence or absence of C/EBPc plasmid

verage beats were checked and intervals set using the first derivative of the tracing. PQ was measured from the onset of P wave to the onset of the QRS wave. QRS duration was measured from the onset of the Q wave to the RNA Purification, cDNA Synthesis and Reverse Transcriptase-Polymerase Chain Reaction Total RNA was extracted from the hearts of control and Fabry KO mice after being homogenized in Trizol, following the instructions of the manufacturer. Total RNA samples were treated with DNase I and reverse-transcribed using random hexamers and the DNA polymerase SuperScriptII. Real-time PCR was carried out on the iCycler using gene-specific primers to quantify the relative abundance of each gene with SYBR Green I as the fluorescent molecule as described. The primers used are listed in Cardiomyopathy in Fabry Mouse 16302825” Model Enzyme Replacement Therapy Mice were inject via tail vein with a single injection of agalsidase-beta at a dose of 3 mg/kg, as previously described; GL3 levels in the heart reached a nadir at 3 weeks following a single intravenous injection of agalsidase-beta at 3 mg/kg, which is the timing and dose of agalsidase-beta used in the present study. Control animals received similar volumes of normal saline. Mice underwent physiologic and echocardiographic assessment prior to injection, and then at 3 weeks following injection, at which point the cardiac GL-3 content was stably reduced by 80%. Statistics Data are expressed as mean 6 SE. Student’s two-tailed t tests were used to compare unpaired data between two groups. If the global test was significant, pair-wise comparisons were performed with a Tuckey-Kramer test. P 0.05 was considered significant. Results Systolic Blood Pressure, Heart Rate, ECG and Cardiac Weight Halofuginone web measurements Systolic blood pressure was lower for male Fabry KO mice than for male wild-type mice . In addition, heart rate was significantly slower in the Fabry KO mice than the WT controls. The measurements of RR intervals with surface ECG recordings showed prolonged RR intervals for Fabry KO mice compared to WT controls. and the standard deviations of the RR intervals were significantly increased in the Fabry KO mice compared to the WT controls after normalization for heart rate. There were no differences in PQ, QRS, or corrected QT intervals. Fabry KO mice had mild diastolic LV dysfunction as depicted with the decrease in Ea velocity, without change in the isovolumic relaxation time. Surface ECGs were obtained in lightly anesthesized mice. Data represent the means 6 SE. Statistical significance was determined by pair-wise comparisons with a Tuckey-Kramer test, or x2 analysis. WT, wild-type; KO, knockout; SDNN, standard deviation of normal RR intervals; APC, %of animals with atrial premature contractions during 10 min recording. P,0.05, P,0.05 by for x2 analysis. 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.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 contraction