Phenotypes Associated with This Genotype

Genotype
MGI:4943692

• Allelic Composition: Gt(ROSA)26Sor^{tm37(H1/tetO-RNAi:Tafazzin)Arte}/Gt(ROSA)26Sor⁺
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6J

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

Defects of cardiac mitochondria and mitochondrion-associated membranes in Gt(ROSA)26Sor^{tm37(H1/tetO-RNAi:Tafazzin)Arte}/Gt(ROSA)26Sor⁺ mice

cardiovascular system

abnormal myocardial fiber morphology ( J:167527 )

• after 8 months, cardiac muscles from doxycycline-treated mice exhibit abnormal mitochondria compared with wild-type mice

• after 8 months, cardiac ventricles from doxycycline-treated mice exhibit poorly arrayed sarcomeres compared to in wild-type mice

abnormal heart left ventricle morphology ( J:167527 )

• at 8 months, doxycycline-treated mice exhibit increased left ventricular diameter and volume both at end diastole and end systole and reduced diastolic thickness of left ventricular posterior wall and interventricular septum compared with wild-type mice

• at 8 months, doxycycline-treated mice exhibit a reduction of left ventricular mass (LV wall volume) compared with wild-type mice

dilated heart left ventricle ( J:167527 )

• at 8 months in a doxycycline-treated mice

decreased cardiac muscle contractility ( J:167527 )

• at 8 months, doxycycline-treated mice exhibit reduced fractional shortening and ejection fraction compared with wild-type mice

cellular

increased mitochondrial number ( J:167527 )

• doxycycline-treated mice exhibit an increase in mitochondria in the heart and muscles compared to in wild-type mice

increased mitochondrial fission ( J:167527 )

• doxycycline-treated mice exhibit an increase in mitochondria in the heart and muscles compared to in wild-type mice

growth/size/body

decreased body weight ( J:167527 )

• in doxycycline-treated mice after 8 months

muscle

abnormal myocardial fiber morphology ( J:167527 )

• after 8 months, cardiac muscles from doxycycline-treated mice exhibit abnormal mitochondria compared with wild-type mice

• after 8 months, cardiac ventricles from doxycycline-treated mice exhibit poorly arrayed sarcomeres compared to in wild-type mice

decreased cardiac muscle contractility ( J:167527 )

• at 8 months, doxycycline-treated mice exhibit reduced fractional shortening and ejection fraction compared with wild-type mice

abnormal sarcomere morphology ( J:167527 )

• after 8 months, cardiac ventricles from doxycycline-treated mice exhibit poorly arrayed sarcomeres compared to in wild-type mice

abnormal skeletal muscle fiber morphology ( J:167527 )

• at 2 months, skeletal muscles from doxycycline-treated mice exhibit increased number of mitochondria with various inner membrane abnormalities compared to in wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Barth syndrome		DOID:0050476	OMIM:302060	J:167527