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Biochemistry Flashcards

6 cards from real USMLE practice questions. Tap to flip, then mark Knew It or Still Learning — missed cards come back until you master them.

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  1. Which enzyme deficiency causes cystinuria by impairing renal tubular reabsorption of dibasic amino acids?

    Answer: Defective cystine transporter (rBAT)

    Cystinuria is caused by a defective cystine/dibasic amino acid transporter (rBAT) in renal tubules and intestine, leading to cystine stones.

  2. A patient on a high-fat diet has elevated chylomicrons and VLDL. Which enzyme is responsible for clearing chylomicrons from the blood?

    Answer: Lipoprotein lipase

    Lipoprotein lipase on capillary endothelium hydrolyzes triglycerides in chylomicrons and VLDL, releasing fatty acids for peripheral uptake.

  3. Which step in heme synthesis occurs in the mitochondria?

    Answer: Formation of delta-aminolevulinic acid (ALA) from glycine and succinyl-CoA

    The first step, formation of ALA from glycine and succinyl-CoA by ALA synthase, occurs in the mitochondrial matrix.

  4. In DNA replication, which enzyme removes the RNA primer and replaces it with DNA?

    Answer: DNA polymerase I

    DNA polymerase I has 5'→3' exonuclease activity to remove RNA primers and 5'→3' polymerase activity to fill the gaps with DNA.

  5. Which amino acid is the primary gluconeogenic substrate released from skeletal muscle during fasting?

    Answer: Alanine

    Alanine is the primary gluconeogenic amino acid exported from skeletal muscle to the liver, representing the glucose-alanine cycle.

  6. Orotic aciduria with megaloblastic anemia but no hyperammonemia suggests deficiency of which enzyme?

    Answer: UMP synthase (UMPS)

    UMP synthase deficiency impairs pyrimidine synthesis, causing orotic acid accumulation and megaloblastic anemia without urea cycle disruption or hyperammonemia.