18 Questions
What is the consequence of fatty acids being oxidized in the liver with the inactivation of PDH?
Conversion of pyruvate to OAA
How is pyruvate carboxylase activated?
By the binding of acetyl CoA
What is a severe consequence of PEPCK deficiency?
Optic atrophy
What leads to the phosphorylation and inactivation of Pyruvate Kinase?
Action of protein kinase A
How is transcription of the gene encoding PEPCK stimulated?
By glucocorticoid–protein complexes
What induces increased production of PEPCK messenger RNA (mRNA)?
Increased cAMP levels
What is the consequence of deficient glucose 6-phosphatase activity in von Gierke disease?
Intracellular accumulation of glucose 6-phosphate
Which enzyme is responsible for converting pyruvate to phosphoenolpyruvate (PEP) in gluconeogenesis?
Pyruvate carboxylase
What is the main clinical feature associated with deficiency of PEPCK (phosphoenolpyruvate carboxykinase)?
Lethargy and seizures
What activates pyruvate carboxylase?
Acetyl CoA
Which enzyme is inhibited by glucagon via cAMP in the pathway shown?
Pyruvate kinase
What induces phosphoenolpyruvate carboxykinase (PEPCK) activity?
Glucagon via cAMP
What is a futile cycle in the context of gluconeogenesis?
A cycle that continuously recycles substrates and products with a net consumption of energy and no useful result
What effect does increased glucagon and decreased insulin have on fatty acids?
Stimulates the release of fatty acids from adipose tissue
Which enzyme catalyzes the conversion of pyruvate to oxaloacetate in gluconeogenesis?
Pyruvate carboxylase
What are the clinical features associated with PEPCK deficiency?
Hypoglycemia and lactic acidosis
Which enzyme is responsible for regulating the conversion of phosphoenolpyruvate to pyruvate in gluconeogenesis?
PEPCK
What is the effect of glucagon on the induction of PEPCK?
Stimulation of PEPCK induction
Test your knowledge on metabolic pathways including the oxidation of fatty acids, production of acetyl CoA, inactivation of PDH, conversion of pyruvate to oxaloacetate (OAA), and activation of enzymes like pyruvate carboxylase and PEPCK.
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