Phenotypes for Pygl MGI:6392248 MGI Mouse

enlarged liver ( J:284765 )

increased liver weight ( J:284765 )

• elevation of liver weight to bod weight ratio

abnormal liver vasculature morphology ( J:284765 )

• 8 of 13 mice show small to large aggregates of mononuclear cells associated with hepatic blood vessels

abnormal liver sinusoid morphology ( J:284765 )

• inconspicuous hepatic sinusoids due to glycogen accumulation and enlargement of hepatocytes

enlarged liver ( J:284765 )

increased liver weight ( J:284765 )

• elevation of liver weight to bod weight ratio

increased liver glycogen level ( J:284765 , J:330539 )

• young and old mice exhibit excessive hepatic glycogen accumulation, with levels about 3-fold higher in old mice than in young mice (J:284765)

• non-fasted and 2 hour-fasted mice show higher accumulation of glycogen in the liver (J:330539)

abnormal hepatocyte morphology ( J:284765 )

• glycogen accumulation in hepatocytes is associated with moderate enlargement to ballooning of hepatocytes

liver fibrosis ( J:284765 )

• 8 of 13 old mice show minimal but occasional mild collagen deposition in the subcapsular, sinusoidal, and/or periportal area of the liver

abnormal liver physiology ( J:284765 )

• old mice exhibit activated hepatic stellate cells in periportal and perisinusoidal areas of the liver

liver inflammation ( J:284765 )

• old mice show mononuclear cell inflammatory infiltrates in hepatic vessels and elevated levels of C-C chemokine ligand 5 and monocyte chemoattractant protein 1 in the liver, indicating inflammation

abnormal homeostasis ( J:284765 )

• 24-hour fasted mice show lower serum lactic acid levels

decreased fasting circulating glucose level ( J:284765 )

• mice exhibit lower blood glucose levels at all tested fasting time points except 24 hours of fasting

hypoglycemia ( J:284765 )

• ketotic hypoglycemia

• however, hypoglycemic seizures are not seen

increased circulating ketone body level ( J:284765 )

• mice exhibit elevated blood ketones at 2, 4, and 6 hours of fasting

decreased circulating cholesterol level ( J:284765 )

• 24-hour fasted mice show lower serum cholesterol levels

decreased circulating triglyceride level ( J:284765 )

• 24-hour fasted mice show lower serum triglyceride levels

increased circulating alanine transaminase level ( J:284765 , J:330539 )

• in old mice (J:284765)

• however, no differences in serum alkaline phosphatase or bilirubin levels (J:284765)

increased circulating aspartate transaminase level ( J:284765 , J:330539 )

• in old mice (J:284765)

increased liver glycogen level ( J:284765 , J:330539 )

• young and old mice exhibit excessive hepatic glycogen accumulation, with levels about 3-fold higher in old mice than in young mice (J:284765)

• non-fasted and 2 hour-fasted mice show higher accumulation of glycogen in the liver (J:330539)

decreased hepatic glucose production ( J:284765 )

• hepatic free glucose levels are decreased

abnormal enzyme/coenzyme activity ( J:330539 )

• mice show reduced hepatic glycogen phosphorylase activity

• however, glycogen phosphorylase activity in muscle is normal