gluconeogenesis

Question 1

enzymes of glycolysis

Answer 1

glucokinase: product inhibition
corresponds to: G6P

Phosphofructokinase-1:allosteric regulation:
inhibited by ATP, citrate
activated by AMP, F-2,6-P
corresponds to: f16BP

pyruvate kinase:in liver, inhibited by phosphorylation
corresponds to:PEPCK

Question 2

enzymes of gluconeogenesis

Answer 2

glucose 6-phosphate: product inhibition
corresponds to: glucokinase

fructose 1,6-bisphosphate
transcription increased by cortisol
inhibited by AMP, F-2,6-P
corresponds to:
Phosphofructokinase-1

phosphoenolpyruvate carboxykinase
key regulatory step
transcriptionally regulated by cAMP and cortisol
corresponds to: pyruvate kinase

Question 3

glycogen storage disease I (GSD I, von Gierke)

Answer 3

glycogen storage disease: fasting hypoglycemia, lactic acidosis, enlarged liver

Question 4

fructose 1,6-bisphosphatase deficiency

Answer 4

rare autosomal recessive disorder
episodic hypoglycemia (when liver glycogen is depleted)