Phenotypes Associated with This Genotype

Genotype
MGI:3849458

• Allelic Composition: Mterf2^{Gt(OST453159)Lex}/Mterf2^{Gt(OST453159)Lex}
• Genetic Background: B6.129S5-Mterf2^{Gt(OST453159)Lex}

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

growth/size/body

decreased body weight ( J:149828 )

• there is a trend of decreased body weight as mice age

• decreased bodyweight is more evident in female than male mice

behavior/neurological

abnormal spatial reference memory ( J:149828 )

• mice fed a ketogenic diet make more mistakes and take longer to find a hidden platform in a water maze after training

impaired exercise endurance ( J:149828 )

• mice run only 75% the distance as controls in an endurance tests

• ketogenic (high-fat, low-carb) diet fed mice only run half the distance as wild-type controls fed the same diet

cellular

increased mitochondrial DNA content ( J:149828 )

• ketogenic diet increases mtDNA 1.5-fold in the brain and 3-fold in the liver

increased skeletal muscle fiber mitochondrial DNA content ( J:149828 )

• 3-fold increase of mtDNA in skeletal muscle

• mtDNA increases to 4.5 fold over controls in skeletal muscle when mice are fed a ketogenic diet

increased mitochondrial number ( J:149828 )

• numbers and size of mitochondria are vastly increased in skeletal muscle

increased mitochondrial size ( J:149828 )

• numbers and size of mitochondria are vastly increased in skeletal muscle

abnormal cellular respiration ( J:149828 )

• lung fibroblasts isolated from mice take significantly longer to grow on a galactose but not glucose media

abnormal tricarboxylic acid cycle ( J:149828 )

• increases in citrate synthase activity

abnormal mitochondrial ATP synthesis coupled electron transport ( J:149828 )

• despite increased mitochondrial mass, ATP levels in skeletal muscle are 60% of controls on a standard diet and just 30% of controls on a ketogenic diet

• mitochondria in skeletal muscles have 30-40% reduced COX activity

• when fed a ketogenic diet, mitochondria from numerous tissues have reduced COX activity

homeostasis/metabolism

impaired exercise endurance ( J:149828 )

• mice run only 75% the distance as controls in an endurance tests

• ketogenic (high-fat, low-carb) diet fed mice only run half the distance as wild-type controls fed the same diet

muscle

increased skeletal muscle fiber mitochondrial DNA content ( J:149828 )

• 3-fold increase of mtDNA in skeletal muscle

• mtDNA increases to 4.5 fold over controls in skeletal muscle when mice are fed a ketogenic diet

muscle weakness ( J:149828 )

• mice have about double the number of falls on a treadmill as do controls

• falls occur about 6-fold more than controls when mice are fed a ketogenic diet

• grip tests confirm the muscle weakness in these mice