UNE Medical Biochemistry Midterm Review STUDY GUIDE 2023.

UNE Medical Biochemistry Midterm Review STUDY GUIDE 2023. 1. Ribose and NADPH: A family presents for genetic counseling. The couple has two female children who are alive and well; however, their infant son recently died in the nursery with severe hemolysis (lysis of red blood cells) and jaundice. The wife recalls she lost a brother in infancy with severe hemolysis, induced after a viral infection. The physician suspects a glucose-6-phosphate dehydrogenase deficiency, this would result in a deficiency in which of the following compounds in red blood cells? 2. Transketolase: A patient presents to the emergency department with lactic acidosis. A deficiency in thiamine (TPP) is suspected and this will be consistent with a decrease in the activity of following enzymes? 3. UDP-Glucose: Galactose 1-phosphate is initially converted to glucose 1-phosphate and requires which of the following compounds? 4. Thiamine diphosphate: Which of the following cofactors is required for transketolase activity? 5. Glyceraldehyde 3-phosphate: Aldolase B facilities a reaction that cleaves fructose 1-phosphate into two 3-carbon molecules that will enter glycolysis as which of the following compounds? 6. Glycolysis: High levels of activity of the non-oxidative portion of the pentose phosphate pathway will directly produce intermediates of which of the following pathways? 7. Glucose 6-phosphate dehydrogenase: The major regulatory step of the pentose phosphate pathway is catalyzed by which enzyme? 8. VLDL particles: Insulin stimulates fatty acid synthesis in the liver. These fatty acids are transported to the adipose tissue as which of the following? 9. Free fatty acids: A young woman goes to work without eating breakfast and skips lunch. In this scenario, which of the following pathways is providing substrates used to generate ATP in the liver? 10. Glucagon: Caffeine is a natural inhibitor of phosphodiesterase. If you were to consume a large amount of coffee you would likely see a prolonged response to which of the following stimulants or hormones? 11. They enhance transcription when bound to receptors: A teenage female presenting with lack of menstruation (amenorrhea) is found to have a dysfunctional steroid receptor characteristic of androgen insensitivity syndrome. Which of the following statements accurately describes steroid hormones? 12. Intracellular receptors: Steroid hormones bind which of the following types of receptors? 13. They are specific to a chemical messenger: Which of the following is a characteristic of all receptors? 1 / 8 UNE Medical Biochemistry Midterm Review 14. Free fatty acids: What is the primary oxidized fuel for skeletal muscle in the fasted state? 15. Cyclic-AMP response element binding protein (CREB): cAMP elicits multiple cellular responses. The effects of cAMP on gene transcription are directly mediated by which of the following DNA binding proteins? 16. GLUT4: An individual is recently diagnosed with Type 2 diabetes. In this individual, hyperglycemia may in part be due to inactivity of which of the following glucose transporters on skeletal muscle? 17. Adenylyl cyclase: Glycogen degradation in the liver can be enhanced by elevated glucagon and the activation of a G±s receptor. The initial step in this cascade involves which of the following second messengers or active enzymes? 18. 2: Which of the following combinations of compounds is required to fully activate pyruvate carboxylase (+ indicates compound is necessary; - indicates compound is not necessary)? 1. Biotin + Acetyl-CoA + Oxaloacetate + Malate - 2. Biotin + Acetyl-CoA + Oxaloacetate - Malate - 3. Biotin - Acetyl-CoA + Oxaloacetate + Malate + 4. Biotin - Acetyl-CoA - Oxaloacetate + Malate + 19. Acetyl-CoA: Which of the following compounds is not a substrate for gluconeogenesis? 20. Pyruvate carboxylase deficiency: A 1-month-old boy is brought to the emergency department in a coma after sleeping through the night and failing to awaken in the morning. He is given intravenous glucose and awakens. Serum levels of pyruvate, lactate, and alanine are elevated, whereas oxaloacetate and aspartate levels are reduced. Which of the following is the most likely diagnosis in this patient? 21. Generation of glucose 1-phosphate to be used for glycogen synthesis: - Which of the following would occur in the liver when glucose levels are high (think insulin is elevated)? 22. The process using lactate from skeletal muscle and red blood cells to generate glucose: Which of the following best describes the role of the Cori cycle? 23. Fructose 2,6-bisphosphate: Which of the following compounds allosterically activates phosphofructokinase-1 (PFK1)? 24. The phosphorylation and inactivation of E1 subunit of PDC (pyruvate dehydrogenase complex): Pyruvate dehydrogenase kinase catalyzes a reaction that results in which of the following? 25. Pyruvate dehydrogenase complex; p