Myh6-R404Q

Fig.1 Detection of Myh6 expression in heart by RT-PCR. 

Wild type and Heterozygous: one band at 456 bp with primers F1/R1(mMyh6) and one band at 123 bp with primers F/R(hGapdh).

Abbr. M, DNA marker; HE, heterozygous; WT, wild type.

Fig.2 mRNA sequencing of Myh6-R404R mice. Sequencing data of RT-PCR products confirmed the presence of mutations in Myh6-R404R mice. 

Abbr. HE, heterozygous; WT, wild type.

Fig.3 Detection of mouse MYH6 expression in Myh6-R404Q mice by WB. 

Heart tissues were collected from wild-type C57BL/6J mice (WT, male, 9 weeks old) and heterozygous hMYH6-R404Q mice (HE, male, 15 weeks old), and then analyzed by western blot with anti-MYH6 antibody (Proteintech, 22281-1-AP). 20 μg total proteins were loaded for western blotting analysis.

Fig.3 Body weight of WT C57BL/6 mice and HE Myh6-R404Q mice.

Abbr.  WT, wild type; HE, heterozygous.

Fig.4 Echocardiographic parameters of HE Myh6-R404Q mice at different ages. 

The results exhibited that the interventricular septal diameter in diastole (IVSd) and systole (IVSs) significantly increased, while the LV internal dimension in diastole (LVIDd) and systole (LVIDs) and the LV end-diastolic volume (LVEDV) and -systolic volume (LVESV) notebly decreased in both male and female Myh6-R404Q mice at the age of 5 months, presenting a phenotype of hypertrophic cardiomyopathy. (n=7-10, Mean±SEM, Unpired t-test or Mann-Whitney test, *P＜0.05, **P＜0.01, ***P＜0.001, ****P＜0.0001, ns: no significance).

Abbr.  WT, wild type; HE, heterozygous; LV, left ventricular.

Fig.5 Echocardiographic parameters of HE Myh6-R404Q mice at different ages. 

The results exhibited that ejection fraction (EF) and fractional shortening (FS) abnormally elevated in both male and female Myh6-R404Q mice at the age of 5 months, as a compensatory response to the impaired ejection function resulting from the reduction in LV volume and diameter. (n=7-10, Mean±SEM, Unpired t-test or Mann-Whitney test, *P＜0.05, **P＜0.01, ***P＜0.001, ****P＜0.0001, ns: no significance).

Abbr.  WT, wild type; HE, heterozygous; LV, left ventricular.