Glucose Metabolism - Correlation Boxes Flashcards
What enzyme does GSD O Dx affect and what does it cause?
Enzyme = glycogen synthase.
Causes inability to elongate glycogen chain
Sx = muscle cramps, hypoglycemia when fastin
What enzyme does GSD IV/Anderson’s disease affect and what does it cause?
Enzyme = glucosyl transferase.
Causes inability of glycogen chain to branch
Sx = hepatosplenomegaly, liver cirrhosis
Glycogen phosphorylase is activated by which enzyme?
Phosphorylase kinase (PK)
Glycogen phosphorylase mutations in the muscle cause what disorder?
GSD V/McArdle’s syndrome
Glycogen phosphorylase mutations in the liver cause what disorder?
GSD VI/Her’s disease
What role does cortisol play under fasting conditions?
Cortisol and amino acids initiate gluconeogenesis
What Dx is associated with hypoxia in the cell? Why?
Dx = lactic acidosis
Cells switch to anaerobic glycolysis leading to increased production of lactic acid
What role does insulin play in expression of glucokinase?
Increased insulin causes activation of glucokinase
What are the differences b/w Type 1 and Type 2 diabetes?
Type 1 = loss of pancreatic ß-cells
Type 2 = insulin resistance followed by ß-cell loss
What enzyme is deficient in hemolytic anemia and what is its mechanism?
Enzyme = G6P dehydrogenase
Causes poor ATP production –> accumulation of Na/H2O in cells –> hemolysis
Why are alcoholics prone to lactic acidosis?
Ethanol uses up NAD+ so there’s an increase in conversion of pyruvate –> lactate to replenish NAD+
Most forms of neonatal lactic acidosis are cause by what?
Inborn Errors of Metabolism (IEMs)
