A 6-month-old girl is brought to the emergency department by her father after he observed jerking movements of her arms and legs earlier in the day. She appears lethargic. Physical examination shows generalized hypotonia. The liver edge is palpable 3 cm below the right costophrenic angle. Her fingerstick glucose shows hypoglycemia. Serum levels of acetone, acetoacetate, and β-hydroxybutyrate are undetectable. Molecular genetic testing shows a mutation in the carnitine palmitoyltransferase II gene. This patient will most likely benefit from supplementation with which of the following?
AThiamine
BCoenzyme A
CMethionine
DTetrahydrobiopterin
EMedium-chain triglycerides
An investigator is studying severely ill patients who experience hypoglycemia and ketonuria during times of fasting. The investigator determines that during these episodes, amino acids liberated from muscle proteins are metabolized to serve as substrates for gluconeogenesis. Nitrogen from this process is transported to the liver primarily in the form of which of the following molecules?
AGlutamate
Bα-ketoglutarate
CAlanine
DArginine
EPyruvate
A scientist is trying to design a drug to modulate cellular metabolism in the treatment of obesity. Specifically, he is interested in understanding how fats are processed in adipocytes in response to different energy states. His target is a protein within these cells that catalyzes catabolism of an energy source. The products of this reaction are subsequently used in gluconeogenesis or β-oxidation. Which of the following is true of the most likely protein that is being studied by this scientist?
AIt is stimulated by epinephrine
BIt is inhibited by glucagon
CIt is inhibited by acetylcholine
DIt is inhibited by cortisol
EIt is stimulated by insulin
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