4. A 7-day-old neonate born to a 28-year-old woman is brought to the office due to progressive lethargy, vomiting, and poor feeding. The mother reports an uneventful pregnancy and perinatal course. She exclusively breastfeeds the
infant and has no medical problems in any of her other children. On examination, the infant is somnolent and dehydrated with decreased muscle tone. Laboratory studies reveal metabolic acidosis with an elevated anion gap,
ketosis, and hypoglycemia. Further evaluation reveals a markedly elevated propionic acid level due to defective conversion of propionyl-CoA to methylmalonyl-CoA. This patient is most likely unable to use which of the following amino acids for energy production?
A. Alanine
B. Aspartate
C. Glutamate
D. Lysine
E. Phenylalanine
F. Valine
5. As part of a research study investigating enzymatic activity in both normal and diseased liver tissue, hepatocytes are isolated from biopsy samples obtained from patients undergoing routine care at a local tertiary referral center.
The cells are homogenized and centrifuged to remove membrane components and organelles. Following subsequent rounds of centrifugation, the remaining supernatant contains only cytosol and cytosolic proteins.
Activity of which of the following enzymes will most likely be detectable in the supernatant of healthy liver cells?
A. 3-Hydroxy-3-methylglutaryl-CoA lyase
B. Omithine transcarbamylase
C. Pyruvate carboxylase
D. Succinate dehydrogenase
E. Transketolase
6. A 43-year-old man prospecting for gold in Arizona becomes stuck in the desert after his truck breaks down. He brought a large supply of water
with him but only a few granola bars as food. After 3 days, he is able to flag down a passing vehicle and obtain transportation to the nearest settlement. During this ordeal, his liver begins to synthesize large quantities of glucose from source molecules such as alanine, lactate, and glycerol. As part of this
process, phosphoenolpyruvate is formed from oxaloacetate in a reaction that requires a specific nucleoside triphosphate as a cofactor. Which of the following reactions directly synthesizes this cofactor?
A. A
B. B
C. C
D. D
E. E
F. F
G. G
H. H