Phenotypes associated with this allele

• Allele Symbol: Tg(Myh6-2A)#Know
• Allele Name: transgene insertion, Kirk Knowlton
• Allele ID: MGI:5569629
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Dmd^tm1.1Know/Dmd^tm1.1Know A1cf^Tg(Myh6-cre/Esr1*)1Jmk/A1cf^+ Tg(Myh6-2A)#Know/0	involves: BALB/c * C3H * C57BL/6 * FVB/N	MGI:5569640
cn2	A1cf^Tg(Myh6-cre/Esr1*)1Jmk/A1cf^+ Tg(Myh6-2A)#Know/0	involves: BALB/c * C57BL/6 * FVB/N	MGI:5905888

Genotype
MGI:5569640

cn1

• Allelic Composition: Dmd^tm1.1Know/Dmd^tm1.1Know; A1cf^{Tg(Myh6-cre/Esr1*)1Jmk}/A1cf⁺; Tg(Myh6-2A)#Know/0
• Genetic Background: involves: BALB/c * C3H * C57BL/6 * FVB/N

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

Inhibition of dilated cardiomyopathy in Dmd^tm1.1Know/Dmd^tm1.1Know Tg(Myh6-2A)#Know/0 A1cf^{Tg(Myh6-cre/Esr1*)1Jmk}/0 mice

cardiovascular system

cardiac fibrosis ( J:207775 )

• less severe fibrosis in tamoxifen-treated mice

increased cardiac muscle contractility ( J:207775 )

• improved cardiac function in tamoxifen-treated mice

abnormal heart echocardiography feature ( J:207775 )

• improved cardiac function in tamoxifen-treated mice

cardiomyopathy ( J:207775 )

• less severe cardiomyopathy in tamoxifen-treated mice

homeostasis/metabolism

decreased susceptibility to injury ( J:207775 )

• tamoxifen-treated mice exhibit less severe cardiomyopathy, cardiac fibrosis and improved cardiac function compared with control mice

muscle

increased cardiac muscle contractility ( J:207775 )

• improved cardiac function in tamoxifen-treated mice

cardiomyopathy ( J:207775 )

• less severe cardiomyopathy in tamoxifen-treated mice

Genotype
MGI:5905888

cn2

• Allelic Composition: A1cf^{Tg(Myh6-cre/Esr1*)1Jmk}/A1cf⁺; Tg(Myh6-2A)#Know/0
• Genetic Background: involves: BALB/c * C57BL/6 * FVB/N

cardiovascular system

abnormal myocardial fiber morphology ( J:128060 )

♂ • irregularity in the spacing between cells in the intercalated discs and irregularity of the sarcolemmal membrane in the hearts of tamoxifen-treated mice

abnormal intercalated disk morphology ( J:128060 )

♂ • myocardial fibers of tamoxifen-treated mice show abnormalities of intercalated discs

abnormal myocardial fiber mitochondrial morphology ( J:128060 )

♂ • cardiac cells from tamoxifen-treated mice show an increase in swollen mitochondria

abnormal myocardial fiber myofibril morphology ( J:128060 )

♂ • cardiac cells from tamoxifen-treated mice show severe cytoskeletal changes such as myofibrillar collapse with a haphazard arrangement and a decrease in myofibril number

increased heart atrium size ( J:128060 )

♂ • biatrial enlargement in tamoxifen-treated mice

increased heart ventricle size ( J:128060 )

♂ • severe ventricular enlargement in tamoxifen-treated mice

cardiac interstitial fibrosis ( J:128060 )

♂ • small amount of interstitial fibrosis in tamoxifen-treated mice

dilated cardiomyopathy ( J:128060 )

♂ • heart weight-to-body weight ratio is increased 22 days after tamoxifen administration

decreased heart left ventricle muscle contractility ( J:128060 )

♂ • decrease in the fractional shortening of the left ventricle of tamoxifen-treated mice

abnormal heart echocardiography feature ( J:128060 )

♂ • increase in left ventricular end-diastolic dimension and end-systolic dimension in tamoxifen-treated mice

heart inflammation ( J:128060 )

♂ • tamoxifen-treated mice show only a small increase in inflammation

homeostasis/metabolism

ascites ( J:128060 )

♂ • in tamoxifen-treated mice

pleural effusion ( J:128060 )

♂ • in tamoxifen-treated mice

immune system

heart inflammation ( J:128060 )

♂ • tamoxifen-treated mice show only a small increase in inflammation

muscle

abnormal myocardial fiber morphology ( J:128060 )

♂ • irregularity in the spacing between cells in the intercalated discs and irregularity of the sarcolemmal membrane in the hearts of tamoxifen-treated mice

abnormal intercalated disk morphology ( J:128060 )

♂ • myocardial fibers of tamoxifen-treated mice show abnormalities of intercalated discs

abnormal myocardial fiber mitochondrial morphology ( J:128060 )

♂ • cardiac cells from tamoxifen-treated mice show an increase in swollen mitochondria

abnormal myocardial fiber myofibril morphology ( J:128060 )

♂ • cardiac cells from tamoxifen-treated mice show severe cytoskeletal changes such as myofibrillar collapse with a haphazard arrangement and a decrease in myofibril number

dilated cardiomyopathy ( J:128060 )

♂ • heart weight-to-body weight ratio is increased 22 days after tamoxifen administration

decreased heart left ventricle muscle contractility ( J:128060 )

♂ • decrease in the fractional shortening of the left ventricle of tamoxifen-treated mice

abnormal sarcolemma morphology ( J:128060 )

♂ • the sarcolemmal membrane is disrupted in myocardial fibers of tamoxifen-treated mice

abnormal sarcomere morphology ( J:128060 )

♂ • myocardial fibers from tamoxifen-treated mice show shortened sarcomeres

abnormal Z line morphology ( J:128060 )

♂ • myocardial fibers from tamoxifen-treated mice show fuzzy Z-lines

respiratory system

pleural effusion ( J:128060 )

♂ • in tamoxifen-treated mice

cellular

abnormal myocardial fiber mitochondrial morphology ( J:128060 )

♂ • cardiac cells from tamoxifen-treated mice show an increase in swollen mitochondria

abnormal myocardial fiber myofibril morphology ( J:128060 )

♂ • cardiac cells from tamoxifen-treated mice show severe cytoskeletal changes such as myofibrillar collapse with a haphazard arrangement and a decrease in myofibril number

cardiac interstitial fibrosis ( J:128060 )

♂ • small amount of interstitial fibrosis in tamoxifen-treated mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
dilated cardiomyopathy		DOID:12930	OMIM:PS115200	J:128060