UNE Biochem Final Exam Questions with Correct Solutions Rated A+

A 3-year-old male is brought to the emergency department by his parents. He is unconscious on admittance and his blood glucose was 15 mg/dl, ( normal 75 - 105 mg/dl). Further testing showed this individual had a deficiency in carnitine. Based on this diagnosis you would also expect which of the following to be true with regards to metabolism: - This patient would have elevated levels of circulating free fatty acids. A 7-year old female is recently diagnosed with type 1 diabetes. Laboratory testing shows elevated urinary glucose and ketones. Blood glucose levels were 250 mg/dL (normal range 90 -120 mg/dL). The increase in blood glucose in this individual is primarily due to increased activity of which of the following enzymes? - Fructose 1,6 bisphosphatase Ketonuria, hyperglycemia and severe acidosis are diagnostic indicators of the presentation of type 1 diabetic ketoacidosis. Which of the following compounds are "ketone bodies" that would be elevated in serum and urine from a patient in this condition? - Beta-Hydroxybutyrate and acetoacetate In the fasted state, fatty acyl-CoA is transported across the mitochondrial membrane. The transport of fatty acids via carnitine palmitoyltransferase I (CPTI) can be directly inhibited by which of the following compounds? - Malonyl-CoA An individual goes to bed around 8 pm and doesn't wake up until 9 am the next morning. During this time, which of the following is the primary oxidizable substrate for skeletal muscle? - Free fatty acids The process of ketogenesis refers to the conversion of acetyl-CoA to which of the following compounds? - beta-hydroxybutyrate A 3-year-old male is brought to the emergency department with hypoglycemia following a brief seizure. His parents put him to bed the night before and he had not eaten much dinner. His urine tested positive for dicarboxylic acids, serum-free fatty acids are elevated and there were no detectable ketones in his blood or urine. In this individual, at the time of presentation his ratio of insulin to glucagon is: - 1 The processes of -oxidation and fatty acid synthesis are regulated in such a manner that neither process is active at the same time. Which of the following enzymes required for fatty acid synthesis is paired with the correct activator? - Acetyl-CoA carboxylase; citrate Following a meal, the ratio of insulin to glucagon will be 1. Which of the following compounds will be elevated to inhibit carnitine palmitoyltransferase I? - Malonyl-CoA Which of the following enzymes require thiamine as a cofactor for activity? (yes, indicates cofactor is required for activity; no, indicates cofactor is not required) - 2: No Yes Yes No Which of the following enzymes is correctly paired with its allosteric inhibitor? - Acetyl-CoA carboxylase: palmitoyl-CoA Which of the following is the regulatory enzyme for fatty acid synthesis? - Acetyl-CoA carboxylase Under conditions when glucose oxidation is elevated, the acetyl-CoA produced will allosterically activate which of the following enzymes leading to an increase in the production of oxaloacetate? - Pyruvate carboxylase Acetyl-CoA Carboxylase will be active when which of the following hormones is elevated? - Insulin During fatty acid synthesis, after the initial step involving acetyl-CoA, the fatty acid chain is elongated by the addition of which of the following compounds? - Malonyl-CoA