Chapter·BiochemistryAmino acid metabolism and disorders

Glucogenic and ketogenic amino acidsDownloads

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1

Researchers are experimenting with hormone levels in mice in fasting and fed states. To test hormone levels in the fed state, the mice are given an oral glucose load and various hormones are measured in a blood sample. Researchers are most interested in the hormone whose blood levels track evenly with C-peptide levels. The hormone the researchers are most interested in is responsible for which of the following actions in the body?

AProtein catabolism

BFatty acid breakdown

CFatty acid synthesis

DKetogenesis

ELipolysis

2

A 4-day-old boy is brought to the physician by his mother because of vomiting, irritability, and poor feeding. Pregnancy and delivery were uncomplicated. Physical examination shows increased muscle tone in all extremities. He appears lethargic. His diapers emit a caramel-like odor. Urine studies are positive for ketone bodies. Supplementation of which of the following is most likely to improve this patient's condition?

ATyrosine

BCysteine

CTetrahydrobiopterin

DThiamine

ELeucine

3

A 20-year-old male is brought by ambulance to the emergency room in extremis. He is minimally conscious, hypotensive, and tachypneic, and his breath gives off a "fruity" odor. An arterial blood gas and metabolic panel show anion gap metabolic acidosis. This patient is most likely deficient in which of the following metabolic actions?

AGlucagon production

BCortisol secretion

CFormation of ketone bodies

DGlucose production

ECellular uptake of glucose

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