UNE FINAL BIOCHEM LATEST EXAM!!

UNE FINAL BIOCHEM LATEST EXAM!! What are the main products of beta oxidation? 1 NADH, 1 FADH, 1 Acetyl CoA So in the case of an 11-year-old girl brought to the emergency department by her father, he's reporting that she's been very thirsty and needing to make frequent strips to the bathroom. Today, she reported lightheadedness during a soccer game and symptoms did not resolve after drinking a power beverage. A rapid dipstick test revealed glucose in her urine and further diagnosis are consistent with a diagnosis of type I diabetes. So evaluation of this girl's liver would reveal an increased rate in which of the following processes? A. Fatty Acid Synthesis B. Glycogenesis C. Glycolysis D. Ketogenesis E. Protein Synthesis D. Ketogenesis A 5 year old female brought to the Emergency Department by her mother, and she's a Type 1 diabetic and has not taken her insulin while she's been away, the child is unresponsive, and we ask the question, which of the following enzymes is phosphorylated and active under these conditions? A. Acetyl- CoA Carboxylase B. Hormone Sensitive Lipase C. Glycogen Synthase D. Phosphofructokinase 2 E. Pyruvate Kinase B. Hormone Sensitive Lipase An individual goes to bed around 8pm and doesn't wake up until 9am the next morning. During this time, which of the following is the primary oxidizable substrate in the skeletal muscle? Free Fatty Acids During the fasted state, lipolysis is facilitated by the action of hormone sensitive lipase. This enzyme requires which of the following modifications for activation? Phosphorylation A well, 2-year-old girl presents to the clinic with symptoms of a viral illness. Her parents report she has had bouts of vomiting and diarrhea over the last 24 hours. This morning they could barely wake her from her crib and she was very weak. Laboratory tests show low blood glucose (hypoglycemia) and elevated liver enzymes. There are no ketones in her urine. The child is hospitalized and glucose is administered by I.V. Additionally laboratory analysis showed elevated medium-chain fatty acyl carnitines in blood and 6-8 carbon dicarboxylic acids in the urine. What is her diagnosis? defect of medium-chain coenzyme A dehydrogenase Under fasting conditions (ratio of insulin to glucagon 1), elevated glucagon will activate which of the following enzymes? Hormone Sensitive Lipase Ketonuria, hyperglycemia and severe acidosis are prime 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 of a patient in this condition? D-3-hydroxybutyrate and acetoacetate In liver, which of the following key inhibitory events ensures that newly synthesized palmitoyl-CoA is not immediately oxidized? Inhibition of acyl-CoA by malonyl-CoA In the fasted state, fatty acyl-CoAs are transported across the mitochondrial membrane. The transport of fatty acids via carnitine palmitoyl transferase I (CPTI) can be directly inhibited by which of the following compounds? Malonyl-CoA An individual is on a hunger strike and has not eaten in 13 days. Which of the following best describes their metabolic state? The brain will be preferentially oxidizing ketones An increase in the ratio of insulin to glucagon will increase the activity of which of the following enzymes (+ indicates activity is increased; - indicates activity is not increased by insulin)? 1.Hormone sensitive lipase 2. Acetyl-CoA carboxylase 3. Phosphofructokinase-2 (PFK2) 4. Glycogen synthase (-) hormone sensitive lipase (+) acteyl- CoA Carboxylase (+) phosphofructokinase -2 (+) Glycogen synthase Fatty acid synthase is a multimeric enzyme complex. Which of the following subunits contains pantothenic acid as a cofactor? Acyl Carrier Protein (ACP) Malate can be converted to pyruvate in the cytosol. Which of the following compounds is directly generated in this conversion and is essential for fatty acid synthesis? NADPH NADPH is essential for fatty acid synthesis and can be generated by which two enzymes? (+ indicates NADPH can be produced by the enzyme; - indicates the enzyme does not produce NAPDH) 1. Glucose 6-phosphate dehydrogenase 2. Malic enzyme 3. Pyruvate dehydrogenase complex (+) Glucose 6-phosphate dehydrogenase (+) Malic enzyme (-) Pyruvate dehydrogenase complex What is the regulatory enzyme for fatty acid synthesis? Acetly-CoA Carboxylase What is the regulated step of lipogenesis in eukaryotes? Carboxylation of acetyl-CoA What is the inhibitor of Acetyl-CoA carboxylase? palmitoyl-CoA An individual consumes a large meal consisting of a cheeseburger and fries. Which of the following intermediates is shuttled out of the TCA cycle to be used as a substrate for fatty acid synthesis? Citrate Which of the following is not required for the transport of newly synthesized fatty acids to the adipose to our extra hepatic tissue? A. Interaction of ApoCII with lipoprotein lipase B. Transport of trigacylglycerols from the liver using VLDLs C. Synthesis of triacylglycerols using glycerool 3-phospjate D. Carnintine to shuttle the Fatty acid out of the mitochondria D. Carnintine to shuttle the Fatty acid out of the mitochondria In reverse cholesterol transport, HDL interacts with the ABC transporters to move cholesterol from the cell to the HDL. This also requires the activity of which of the following enzymes? LCAT Which of the following proteins is responsible for the transfer of ApoB48 to nascent chylomicrons before secretion into circulation? MTP A 12-year-old male is brought to his primary care physician. His parents are concerned with his recent decline in school work due to persistent blurred vision. On physical exam, planar xanthomas are noted and laboratory testing reveals extremely low plasma HDL. This presentation would be consistent with a loss of which of the following apoproteins? ApoA Nascent chylomicrons are synthesized in which of the following organs? Intestine A 5-year old presents to her primary care physician. Her mother is concerned due to persistent diarrhea which is worse when eating fatty foods. The child also has some clinical signs of a deficiency in vitamins A, D, E and K (fat soluble vitamins). Based on this information the child likely has a deficiency in the ability to synthesize which of the following lipoproteins? Chylomicrons Shortly after meal high in fat, you would expect an elevation of which of the follow lipoproteins in circulation? Chylomicrons The interaction of chylomicrons with lipoprotein lipase is stimulated by which of the following Apo proteins? ApoCII A 16-year-old woman presents to her primary care physician for a follow up visit. At her initial visit, 3 months ago, her serum cholesterol was 255 mg/dL (above normal). Today her serum triglycerides are reported to be 260 mg/dL (above normal) . She finds this very disappointing as she started a strict dietary regime reducing all fats and cholesterol, replacing them with a high carbohydrate diet.