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This comprehensive study guide for une's biochemistry final exam covers key concepts and metabolic pathways, providing a valuable resource for students preparing for the exam. It includes multiple-choice questions with answers, covering topics such as lipid metabolism, amino acid metabolism, nucleotide metabolism, and more. The guide is designed to help students solidify their understanding of essential biochemical processes and prepare for the challenges of the final exam.
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A comatose infant is brought to the ER. In the course of the examination, plasma ammonia was found to be elevated 20-fold over normal values. Urine orotic acid and uracil were both greater than normal. A defect of which of the following enzymes is the most likely diagnosis?
ß synthase. In this individual which of the following amino acids could potentially become essential? - ANSCysteine Phenylketonuria can be caused by an enzymatic deficiency or it can present due to a deficiency in which of the following required metabolic cofactors? - ANSTetrahydrobiopterin (BH4) A 17-year old female presents to her primary care and is diagnosed with a Vitamin B (cobalamin) deficiency. In this individual, which of the following reactions would be compromised? - ANSConversion of methyl malonyl-Co A to succinyl-CoA A 15-year-old female presents to her pediatrician with concerns over her inability to sleep, increased feelings of depression and generalized fatigue. A full laboratory work-up is ordered and based on these findings she is diagnosed with a melatonin deficiency. Based on this information, metabolism of which of the following amino acids is likely impaired? - ANSTryptophan A 52-year- old male sought medical help complaining about progressive appearance of hyperchromic (hyper pigmented) papules on the edge of the second finger of both hands. He also complained about darkening of urine. Incisional biopsy of one of the spots on his hands and urine sample tested positive for or homogentisic acid. Based on these findings a diagnosis of alkaptonuria is made. This diagnosis is consistent with a deficiency in which of the following enzymes? - ANSHomogentisate oxidase Methotrexate is a potent anticancer agent that starves dividing cells of deoxyribonucleotides through direct inhibition of which of the following processes? - ANSMetabolism of folic acid A 47-year-old man complains of pain in the joints of his left big toe, which are obviously swollen and tender. The pain has been chronic but became intolerable the day after Thanksgiving when he had a large meal and several glasses of red wine. Based on this information a diagnosis of gout is made. Which of the following is involved in the pathophysiology of this patient's condition? - ANSElevated uric acid Which of the following is the common intermediate for purine synthesis? - ANSInosine monophosphate The ribonucleotide reductase is important for converting ribose bases to the deoxy form. This conversion is coupled with the oxidation of which of the following compounds? - ANSThioredoxin In pyrimidine synthesis, carbamoyl phosphate synthetase II (CPSII) can be allosterically activated by which of the following compounds? - ANS5-phosphoribosyl 1-pyrophosphate (PRPP) Which of the following enzymes is used directly in the synthesis of dTMP (thymidine)? - ANSThymidylate synthase The regulation of PRPP synthetase is critical in the production of both purines and pyrimidines. This enzyme is inhibited under which of the following conditions? - ANSWhen both GDP and ADP are elevated A 4-year-old girl presented to the clinic with megaloblastic anemia and failure to thrive. Urinalysis showed significant sediment with extremely high levels of orotic acid. A
Which of the following compounds or substrates is required for the synthesis of tyrosine? (+ indicates the compound is required; - indicates the compound is not required) - ANS A 2-day-old infant test positive for hyperphenylalaninemia. This is most likely caused by a heritable defect in which of the following enzymes? - ANSPhenylalanine hydroxylase You and a friend are being chased by a bear. Under these conditions levels of epinephrine are increased due to metabolism of which of the following amino acids? - ANSTyrosine In pyrimidine synthesis, uridine triphosphate (UTP) can feedback and inhibit which of the following enzymes? - ANSCarbamoyl phosphate synthase II Which of the following characteristics distinguish most RNA molecules from DNA? - ANSUridine base on RNA, whereas a thymidine base on DNA Which of the following lipoproteins distributes dietary lipids? - ANSChylomicron The interaction fo chylomicrons with lipoprotein lipase is stimulated by which of the following App proteins? - ANSApoCII Nascent chylomicrons are synthesized in which tissue? - ANSIntestine Bile salts are synthesized from cholesterol and are used for the emulsification of dietary lipids. What is the primary fate of bile salts during digestion? - ANSThey are reabsorbed in the ileum Nascent chylomicrons interact with HDL particles and obtain which fo the following Apo proteins? - ANSApoCII and ApoE The activity of pancreatic lipase is inhibited by which of the following compounds? - ANSBile salts Which fo the following statements regarding liid digestion and absorption is true? A. The products of lipid digestion travel in the circulatory system in the form of micelles. B. The products of lipid digestion diffuse into capillaries in the vicinity of the intestinal epithelium. C. Pancreatic lipase emulsifies lipids. D. The products of lipid digestion are resynthesizes into triacylglycerols in the intestinal epithelial cells. e. In the absence of bile salts, no digestion of lipids would occur. - ANSThe products of lipid digestion are resynthesizes into triacylglycerols in intestinal epithelial cells. Under fasting conditions, elevated glucagon will activate which of the following enzymes? - ANSHormone sensitive lipase VLDL particles are characterized by which of the following apoproteins? - ANSApoB An increase in insulin leads to which of the following metabolic shifts? - ANSAcetyl CoA carboxylase will become dephosphorylated and active. Biotin is a cofactor required for which of the following enzymes? - ANSA and B (Acetyl CoA carboxylase and Pyruvate carboxylase) The first step in fatty acid synthesis is the formation of ________ from acetyl CoA and carbon dioxide. - ANSMalonyl CoA Insulin increases the activity of all of the following enzymes except - ANSHormone sensitive lipase
Which fo the following is the regulatory enzyme for fatty acid synthesis? - ANSAcetyl CoA carboxylase Which fo the following is the regulated step of fatty acid synthesis in eukaryotes? - ANSCarboxylation of acetyl CoA Under conditions of starvation, all of the following are true except - ANSMalonyl CoA will be elevated Which of the following proteins is responsible for the transfer of ApoB48 to nascent chylomicrons before secretion into circulation? - ANSMicrosomal transfer protein (MTP) The process of beta-oxidation generated all of the following compounds except - ANSNADPH During the fasted state, lipolysis is facilitated by the action of hormone sensitive lipase. This enzyme requires which of the following post - translational modification for activation? - ANSPhosphorylation Adiponectin is secreted from adipocytes. One of the primary roles of adiponectin is to activate the MAPK pathway in skeletal muscle which enhances the activity of which of the following pathways? - ANSBeta-oxidation When the process of 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? - ANSPyruvate carboxylase The process of ketogenesis refers to the conversion of acetyl CoA to which of the following compounds? - ANSBeta-hydroxybutyrate The transport of newly-synthesized fatty acids to the adipose requires all fo the following except - ANSCarnitine to shuttle the fatty acids out of the mitochondria In the case of deficiency of biotin, which of the following enzymes required for fatty acid synthesis will be impaired? - ANSAcetyl CoA carboxylase Which of the following compounds is the substrate for the synthesis of cortisol? - ANSCholesterol Under cellular conditions when cholesterol is low, which of the following is a true statement regarding the sterol response element binding protein? - ANSIt will be associated with the sterol response element (SRE) in the DNA HDL particles are characterized by the presence of which apoprotein? - ANSApoA Which of the following is the regulatory enzyme in cholesterol synthesis? - ANSHMG CoA reductase Which of the following lipoproteins is responsible for the transport of cholesterol to peripheral tissue? - ANSLDL Which of the following enzymes is responsible for moving cholesterol from HDL to VLDL?
Increase in ethanol consumption leads to an increase in the ratio of NADH/NAD+. This promotes the conversion of pyruvate to which fo the following compounds? - ANSLactate The oxidation of ethanol increases the ratio of NADH/ NAD in both the mitochondria and the cytosol. This leads to the inhibition of which of the following enzymes in the TCA cycle? - ANSIsocitrate dehydrogenase Several friends went out Saturday evening to celebrate an exam. They skipped dinner and decided to head straight to the bar. The next morning one of the individuals was found laying unconscious at the bottom of the stairs. He was rushed to the emergency department and blood glucose levels of 30mg/ dL (normal range 100-120mg/ dL). At the time of presentation, which of the following would be consistent with this individuals signs and symptoms (when compared to a normal individual)? - ANSIncrease lactate levels Which of the following is the primary metabolic fate of acetate generated from ethanol metabolism? - ANSIt is converted to acetyl CoA by peripheral tissues Both gluconeogenesis and fatty acid beta oxidation are required for homeostasis in the fasted state. Which of the following statements is false with regards to how these pathways interact?
Pyruvate carboxylase is allosterically by which of the following compounds? - ANSAcetyl CoA Which lipoprotien is responsible for the process of reverse cholesterol transport (the movement of cholesterol from the peripheral tissues back to the liver)? - ANSHDL Which of the following enzymes is responsible for the conversion of ethanol to acetaldehyde in the cytosol? - ANSAlcohol dehydrogenase Gluconeogenesis generates glucose 6-phosphate. Which of the following is the primary fate of this compound generated by this process? - ANSDephosphorylation and release into circulation Which of the following apoproteins is present on an LDL particle and required for uptake by peripheral tissues? - ANSApoB Which enzyme is responsible for transferring triacylglycerol from VLDL to HDL in exchange for cholesterol? - ANSCEPT (cholesterol ester transferase protein) Which of the following is the full complement of apoproteins present on VLDLs? - ANSApoB100, ApoCII, ApoE What is the primary role of LCAT (Lecithin-cholesterol acyltransferase)? - ANSTo esterify cholesterol transported by HDL Several hours after a high carbohydrate meal, the newly synthesized triacylglycerols in the liver will be packaged into which of the following lipoproteins? - ANSVLDL Which of the following apoproteins is required for activation of lipoprotein lipase? - ANSApoCII Which of the following lipoproteins is responsible for carrying cholesterol from peripheral tissues (i.e skeletal muscle) back to the liver? - ANSHDL Which of the following lipoproteins is responsible for the transport of dietary triacylglycerols in the blood? - ANSChylomicrons HMG CoA reductase is highly regulated within the cell. Which of the following will reduce the activity of HMGCoA reductase? - ANSPhosphorylation by a protein kinase Which of the following molecules is not synthesized using cholesterol as a substrate? - ANSInsulin The processes of beta-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 β oxidation is paired with the correct inhibitor? - ANSCarnitine palmitoyl transferase I (CPTI); malonylCoA Elevated levels of acetyl CoA can be converted into ketone bodies and can potentially be used as fuel by all of the following tissues except - ANSLiver AcetylCoA Carboxylase will be active when which of the following hormones is elevated? - ANSInsulin Which of the following compounds can inhibit the actions of pancreatic lipase? - ANSBile salts Nascent chylomicrons originate from which of the following tissues? - ANSIntestine Which of the following enzymes generates NADPH? - ANSMalic enzyme
Under fasted conditions which process provides a primary oxidizable substrate for the brain?
D. Glycogen synthase - ANSGlutaminase Which of the following enzymes is correctly paired with its activator? A. N-acetylglutamate synthtase; arginine B. Carbamoyl phosphate synthetase I; ATP C. Glutaminase; citrate D. arginase; NADH - ANSN-acetylglutamate synthtase; arginine In the case of a urea cycle deficiency, which of the following amino acids may become essential? - ANSArginine The glucose alanine cycle is a method of transferring ammonia from the skeletal muscle to the liver. In this process the non-toxic carrier of ammonia is which of the following compounds? - ANSAlanine Pepsinogen is activated by which of the following mechanisms or enzymes? - ANSA change in pH catalyzes the activation of pepsinogen to pepsin The process of autophagy is defined as which of the following processes? - ANSRecycling of proteins under conditions of cellular need or starvation When is the process of autophage most likely occurring within the cell? - ANSWhen there is low energy in the cell; ratio of AMP/ATP is > The pancreatic zymogen trypsinogen is activated by which of the following enzymes or compounds? - ANSEnteropeptidase Which of the following reactions takes place in the mitochondria? - ANSThe synthesis of carbamoyl phosphate Unlike many of the other metabolic pathways the urea cycle can be active under which of the following conditions? - ANSBoth the fed an the fasted state Which of the following will contribute to elevated triglycerides in individuals with type 2 diabetes? - ANSReduced activity of lipoprotein lipase Which of the following GLUT transporters facilitates glucose uptake in skeletal muscle? - ANSGLUT 4 Alanine can be shuttled from the skeletal muscle to the liver where it is deaminated by which of the following enzymes? A. alanine dehydrogenase B. alanine aminotransferase C. glutamine dehydrogenase D. glutaminase - ANSAlanine aminotransferase
A 25-year old female is diagnosed with sandfly fever. The combination of the infection and elevated cortisol will increase protein catabolism. In this individual you would predict nitrogen balance to be - ANSNegative Which of the following GLUT transporters is used by skeletal muscle when insulin is high? - ANSGLUT 4 A patient is experiencing uncontrolled diabetes mellitus with an overproduction of β- hydroxybutyric acid (ketones) and deep breaths (Kussmauls). Which statement is false? - ANSHer blood pH will be alkaline (pH >7.4) Samantha is a 24-year old female with type 1 diabetes. She could not recall whether she took her insulin injection after dinner and accidentally administers an additional dose of insulin. Twenty minutes later she feels dizzy and tests her blood glucose which was 55 mg/dl, (normal 75 - 105 mg/dL). The presentation of hypoglycemia is initially and most immediately due to - ANSincreased glucose uptake by GLUT 4 A 16-year-old girl complains that she often feels light-headed and dizzy just before lunch at school. She tells you that she often does not eat breakfast. She almost always feels better after lunch. You explain to her that she is experiencing hypoglycemia. Which of the following is true under the fasting conditions described above? - ANSCortisol is increased A 16-year-old girl complains that she often feels light-headed and dizzy just before lunch at school. She tells you that she often does not eat breakfast. She almost always feels better after lunch. You explain to her that she is experiencing hypoglycemia. Under these conditions, you would expect - ANSGlycogen phosphorylase kinase to be phosphorylated and active Thyroid hormone is classified as a peptide hormone but it signals in a manner more closely related to which of the following hormones? A. cortisol B. Insulin C. epinephrine D. Growth hormone - ANSCortisol Which of the following hormones will inhibit the release of growth hormone from the anterior pituitary? A. Insulin B. Growth hormone releasing factor C. Somatostatin
D. Growth hormone - ANSSomatostatin Release of ACTH, from the pituitary, can be inhibited by which of the following hormones? A. Cortisol B. Insulin like growth factor C. Growth hormone D. Epinephrine - ANSCortisol When cortisol is elevated which of the following processes is enhanced? - ANSHepatic protein synthesis (i.e. protein translation) TSH secreted from the pituitary stimulates the release of T3 and T4 from the thyroid. Which of the following hormones provides negative feedback to the pathway? A. T B. TRH C. Cortisol D. T4 - ANST When thyroid hormone is bound to its receptor this will have which of the following impacts on DNA accessibility? - ANSIncrease In a patient with decreased secretion of growth hormone due to a pituitary tumor, which of the following would be true? - ANSIncreased GHRH from hypothalamus, diminished IGF- 1 secretion from liver The thyroid is unique as it can store thyroid hormone within the colloid space. Within the space the thyroid hormone is primarily stored in which of the following forms? - ANSAs part of the thyroglobulin protein Which of the following processes does epinephrine enhance? - ANSHepatic glycogenolysis Which two hormones bind intracellular receptors that can function as transcription factors? - ANSCortisol and T In the absence of thyroid hormone, where is the thyroid hormone receptor located? - ANSWithin the nucleus bound to DNA The catecholamines are part of the body's fight or flight response and will increase many different responses. Which of the following is not increased by release of catecholamines? - ANSRelease of insulin Which of the following hormones signal through Tyrosine Receptor Kinase (TRK) receptors? A. aldosterone
C. Trypsin D. H+ - ANSEnteropeptidase Which of the following is an insulin insensitive glucose transporter on the liver? - ANSGLUT Which of the following enzymes is the regulatory step in the urea cycle? - ANSCarbamoyl phosphate synthetase I (CPS I) Which enzyme initiates the activation of pancreatic zymogens required for protein digestion? A. chymotrypsin B. trypsinogen C. HCL D. enteropeptidase - ANSEnteropeptidase Manny is a 3-year-old male who is brought to the emergency room by his parents. He is unconscious on admittance and has a history of multiple hospital admissions with episodes of depressed mental status or coma and hypoglycemia. 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 - ANSThis patient would have elevated levels of circulating free fatty acids The release of insulin from the pancreas is inhibited by which of the following hormones? A. growth hormone releasing factor B. glucagon C. somatostatin D. growth hormone - ANSSomatostatin In liver, which of the following key inhibitory events ensures that newly synthesized palmitoyl-CoA is not immediately oxidized? - ANSInhibition of carnitine-palmitoyl transferase-I (CPT-I) by malonyl-CoA. An 10-year-old boy is brought to the emergency department by his father. He reports his son has recently been very thirsty and needing to make frequent trips to the bathroom. Physical examination revealed a thin boy, in the 30th percentile for height and weight. A rapid dipstick test revealed glucose in his urine. Evaluation of this boy's blood would reveal decreased levels of which of the following compounds or hormones? - ANSInsulin
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 present in her urine. The child is hospitalized and glucose is administered by I.V. Additional laboratory analysis showed elevated medium-chain fatty acyl carnitines in blood and 6-8 carbon dicarboxylic acids in the urine. Which of the following abnormalities is the most likely diagnosis for this child? - ANSDefect of medium-chain coenzyme A dehydrogenase ATP can be generated by many metabolic pathways in the body. Which of the following processes generates the most ATP? - ANSFatty acid β-oxidation The process of ketogenesis refers to the conversion of acetyl-CoA to which of the following compounds? - ANSD- 3 - hydroxybutyrate or β-hydroxybutyrate An 11-year-old girl is brought to the emergency department by her father. He reports she has recently been very thirsty and needing to make frequent trips to the bathroom. At her soccer game today, she complained of lightheadedness and the symptoms did not resolve even after drinking a power beverage. A rapid dipstick test revealed glucose in her urine and further diagnostics were consistent with a diagnosis of type 1 diabetes. Evaluation of this girl's liver would reveal an increased rate of which of the following processes? - ANSKetogenesis 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 in the skeletal muscle? - ANSFree fatty acids 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? - ANSD- 3 - hydroxybutyrate and acetoacetate 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) - ANS1 Glucose 6P + Malic + PDC - Which of the following is the regulatory enzyme for fatty acid synthesis? - ANSAcetyl-CoA carboxylase 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? - ANSNADPH 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? - ANSCitrate A defect in the thioesterase subunit of fatty acid synthase would impair which of the following activities of the enzyme complex? - ANSLiberation of newly assembled palmitate from the enzyme 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? - ANSPyruvate carboxylase
Which of the following best describes the mechanism by which cholesterol is eliminated? - ANSCholesterol is used to synthesize secondary bile acids that are eliminated in the feces In the reaction catalyzed by cholesteryl ester transfer protein (CETP), the VLDL particle receives which of the following compounds from HDL particles? - ANSCholesteryl-ester 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? - ANSLecithin cholesterol acyltranferase (LCAT) Which of the following is not a product of β-oxidation? - ANSNADPH During the fasted state, lipolysis is facilitated by the action of hormone sensitive lipase. This enzyme requires which of the following modifications for activation? - ANSPhosphorylation Which of the following statements is consistent with nitrogen transport in the fasted state? - ANSThe urea cycle will be active The urea cycle takes place in which of the following subcellular locations? - ANSThe cytosol and the mitochondria Which of the following enzymes is correctly paired with its activator? - ANSN- acetylglutamate synthtase; arginine A deficiency in which of the following enzymes will not result in an increase in orotic acid? - ANSN-acetylglutamate synthetase Which of the following is the primary role of glutaminase in the liver? - ANSTo free an amine group from glutamine Which of the following enzymes is likely to be active in both the fed and fasted states? - ANSGlutaminase Which of the following best describes the process of transamination? - ANSThe transfer of an amino group from an amino acid to a keto-acid. Glucogenic amino acids that are used used in the process of gluconeogenesis and will be metabolized to all of the following compounds except: - ANSAcetyl-CoA The glucose-alanine cycle is a method of transferring ammonia from the skeletal muscle to the liver. In this process the non-toxic carrier of ammonia is which of the following compounds? - ANSAlanine A newborn infant was resuscitated at birth and was found to be neurologically compromised, with marked flaccidity (lack of muscle tone), and cardiac insufficiency. Glutamine was largely absent from the serum and ammonia levels were excessively elevated and urea production was otherwise not impaired. Based on this information which of the following enzymes is likely deficient in this infant? - ANSGlutamine synthetase Which of the following enzymes is regulated by N-acetylglutamate (NAGs)? - ANSCarbamoyl phosphate synthetase I (CPS I) A deficiency in B6 (pyridoxal phosphate) would negatively impact which of the following pathways: