A 4-month-old boy is being evaluated for seizures, psychomotor retardation, and hypotonia.

Work up reveals elevated serum levels of lactate, alanine, pyruvate and keto acids. Based on the clinical presentation,pyruvate carboxylase activity is measured using fibroblasts from a skin biopsy and is found to be markedly decreased. This enzyme is normally used to directly synthesize which of the following molecules ? a) Pyruvate b) Oxaloacetate c) Malate d) Acetyl co A e) - Keto glutarate 2) A 34-year-old woman presents with central obesity, relatively thin extremities and purple stria on her abdomen. Further work up reveals an excessive serum cortisol level and a blood sugar level of 258 mg/dl. Which of the following is the most likely cause of her hyperglycemia ? a) Excessive consumption of processed carbohydrates b) A pancreatic adenoma secreting ACTH and glucagon c) Cortisol induced inhibition of insulin d) Glucorticoids cause induction of Phosphoenolpyruvate carboxy kinase gene e) Increased gluconeogenesis due to increased provision of substrates through fatty acid oxidation. 3) A 56-year-old, obese man complains of polydipsia, polyuria and fatigue. A glucose tolerance test confirms the diagnosis of diabetes. He is placed on Metformin, which works by which of the following mechanisms ? a) Inhibiting hepatic gluconeogenesis b) Increasing glucagon level c) Increasing cellular responsiveness to circulating insulin d) Stimulating the release or preformed insulin e) Replacing the need for endogenous insulin

4) An enzyme used in both glycolysis and gluconeogenesis is: a) 3-phosphoglycerate kinase. b) Glucose 6-phosphatase c) Hexokinase d) Phosphofructokinase-1 e) Pyruvate kinase 5) Which one of the following statements about gluconeogenesis is false? a) For starting materials, it can use carbon skeletons derived from certain amino acids. b) It consists entirely of the reactions of glycolysis, operating in the reverse direction. c) It employs the enzyme glucose 6-phosphatase. d) It is one of the ways that mammals maintain normal blood glucose levels between meals. e) It requires metabolic energy (ATP or GTP). 6) All of the following enzymes involved in the flow of carbon from glucose to lactate (glycolysis) are also involved in the reversal of this flow (gluconeogenesis) except: a) 3-phosphoglycerate kinase. b) Aldolase c) Enolase d) Phosphofructokinase-1 e) Phosphohexoseisomerase 7) In humans, gluconeogenesis: a) can result in the conversion of protein into blood glucose. b) helps to reduce blood glucose after a carbohydrate-rich meal. c) is activated by the hormone insulin

d) is essential in the conversion of fatty acids to glucose. e) requires the enzyme hexokinase. 8) Which of the following substrates cannot contribute to net gluconeogenesis in mammalian liver? a) Alanine b) Glutamate c) Palmitate d) Pyruvate e) -Keto glutarate. 9) A 6-year-old boy begins playing soccer in a community league. After his first game of the year, he is brought to see his pediatrician because of his severe muscle cramps and blood in urine. He is subsequently found to be having deficiency of lactate dehydrogenase. This enzyme is important in which of the following conversions ? a) Pyruvate to Acetyl co A b) Pyruvate to Oxaloacetate c) Pyruvate to Alanine d) Pyruvate to Phosphoenol pyruvate e) Pyruvate to lactate 10) A new-born infant is found to be having persistent hypoglycemia, despite decreased feeding intervals. The child is also irritable with a moderate degree of hepatomegaly. He is found to have a normal level of muscle fructose 1,6 bisphosphatase but decreased level of liver isoform. Which of the following statement is true of fructose 1,6 bisphosphatase? a) It is induced by Adenosine mono phosphate (AMP) b) It is induced by Insulin c) It is inhibited by Fructose 2,6 bisphosphate d) It is induced in the fed state e) It is inhibited during fasting.

11) A 14-year-old high school girl who is extremely conscious about her appearance has gone a full day fasting to fit in to a dress she intentionally brought a size smaller than her actual size for a dance party. Which of the following organs/tissues contributes to the glucose that is being synthesized through gluconeogenesis ? a) Spleen b) Red blood cells c) Skeletal muscle d) Liver e) Brain 12) An 8 -year-old boy presents with frequent episodes of weakness, accompanied by sweating and feelings of dizziness. Physical examination is remarkable for palpably enlarged liver and kidneys. Blood biochemistry reveals lactic acidemia. The patient is diagnosed with deficiency of glucose-6-phosphatase, which is normally only expressed ina) Liver and muscle b) Liver and brain c) Erythrocytes d) Liver and adipose tissues e) Liver and kidney. 13) A 32-year-old body builder has decided to go on a diet consisting of egg whites to ensure only proteins for muscle growth. After a few weeks he experiences decreased energy and is found to be hypoglycemic. A nutritionist tells the patient that he most likely has the deficiency of vitamin Biotin. Which of the following enzymes is unable to catalyze its step in synthesizing glucose from pyruvate? a) Pyruvate carboxylase b) Phospho enol pyruvate carboxy kinase c) Glucose-6-phosphatase d) Fr 1,6 bisphosphatase e) Phosphoglycerate kinase.

14) In an individual at rest, who has fasted for 12 hours, which of the following occurs ? a) Gluconeogenesis is the major process by which blood glucose is maintained b) Liver glycogen stores are depleted c) Glycogen synthase is activated in liver d) Adenylate cyclase is inactivated in liver e) Phosphorylase, pyruvate kinase and glycogen synthase are phosphorylated in liver. 15) Which of the following is a common intermediate in the conversion of glycerol and lactate to glucose ? a) Pyruvate b) Oxaloacetate c) Malate d) Glucose-6-phosphate e) Phosphoenolpyruvate. 16) In the conversion of pyruvate to glucose during gluconeogenesis, a) Biotin is required b) CO2 added in one reaction, appears in the final product c) Energy is used up in the form of GTP d) All the reactions take place in the cytosol e) PDH is the allosteric enzyme used. 17) Which of the following glycolytic enzymes is used in gluconeogenesis ? a) Glucokinase b) Phosphofructokinase-1 c) Aldolase B d) Phosphoglycerate kinase

e) Pyruvate kinase. 18) The conversion of pyruvate to fructose 1,6 bisphosphate by the gluconeogenic pathway involves which of the followings ? a) Requires lactate dehydrogenase b) Requires hexokinase c) Produces 2 ATP and 2GTP d) Requires 4 ATP and 2 GTP e) Acetyl co A is not required to activate this pathway. 19) A high ratio of insulin to glucagon can: a) Promote gluconeogenesis b) Promote glycogenolysis c) Found in Type 1 Diabetes Mellitus d) Occurs during fasting state e) Promotes synthesis of Glycogen 20) Which of the followings is a precursor for gluconeogenesis ? a) Glycogen b) Glycerol c) Glucagon d) Galactose e) Glucose-1- P.

Answers- 1)-b , 2)-d, 3)-a, 4)-a, 5)-b, 6)-d, 7)-a, 8)-c, 9)-e, 10)-c, 11)-d, 12)-e, 13)-a, 14)-e, 15)-d, 16)-a ,17)-d, 18)-d, 19)-e, 20)-b.

1) An infant presents with an enlarged heart, muscle weakness, and hypoventilation. She is diagnosed with glycogen storage disease type II, a disease-causing, abnormal glycogen storage in the heart, skeletal muscle and respiratory muscles. Glycogen synthase is the regulatory enzyme for glycogen synthesis. It adds glucose residues to the nonreducing ends of a glycogen primer from: a) Glucose-1-P b) Glucose-6-P c) UDP- Glucose d) UTP e) ATP. 2) A 30- year-old male presents with severe muscle cramps. He is found to have muscle glycogen phosphorylase deficiency(Mc Ardles disease, Glycogen storage type 5).Glycogen phosphorylase degrades glycogen to producea) Glucose b) Glucose-1-P c) Glucose-6-P d) UDP Glucose e) Glycogen Primer 3) A 15-year-old type 1 diabetic faints after injecting himself with insulin. He is administered Glucagon and rapidly recovers consciousness. Glucagon induces activity of: a) Glycogen synthase b) Glycogen phosphorylase c) Glucokinase d) Hexokinase e) UDP Glucose pyrophosphorylase. 4) A 30-year-old presents with intractable vomiting and inability to eat or drink for the past 3 days. His blood glucose level is normal. Which of the following is most important for maintenance of blood glucose ?

a) Liver b) Heart c) Skeletal muscle d) Lysosome e) Spleen. 5) Shortly after birth, an infant presents with tremors, irritability and seizures. His blood glucose is profoundly low, and he is fed through a nasogastric tube. he is diagnosed with glycogen storage disease type I, a disease that causes excessive build up of liver glycogen, resulting in abnormally low blood glucose level. In glycogen, the linkage at branch points is: a) -1,4 b) -2,3 c) -1,6 d) -1,4 e) -2,3. 6) A 15-year-old male presents with increased thirst, hunger, urination, and weight loss. His fasting blood glucose level is 400 mg/dl and is diagnosed with type 1 diabetes mellitus. What is the reason for this patients inability to maintain a normal blood glucose level ? a) Decreased uptake of glucose by peripheral cells b) Abnormal response to glucagon c) Decreased glucagon to insulin ratio d) Decreased glucose output by the liver e) Increased ketone body production. 7) Which enzyme is not present in muscle ? a) Phosphorylase b b) Hexokinase

c) Glucose-6-phosphatase d) Glycogen synthase e) Lactate dehydrogenase 8) Glycogenolysis is best described by which of the following statements ? a) It involves enzymes cleaving , 1-4 glycosidic linkage b) Requires activation of glycogen synthase c) Requires a dual action enzyme -1,4 glycosidase and transferase d) Uses ATP to produce glucose-1-P e) Requires inactivation of phosphorylase kinase. 9) Glycogenesis from Glucose-1-P requires which of the followings ? a) Phosphoglucomutase b) Uridine triphosphate (UTP) c) -1,6 glucosidase d) Glycogen primer e) Uridine diphosphate 10) In glycogen, glucose residues form a straight chain via which of the followingsa) -1,4 linkages b) -1,6 linkages c) -1,4 linkages with glycogenin at the non reducing end d) -1,6 linkages with UDP glucose at any end e) straight chain occurs at 3 residues apart 11) In the liver Epinephrine induced glycogenolysis : a) Requires UDP glucose pyrophosphorylase

b) Requires Phosphoglucomutase c)Requires inactivation of phosphorylase a d) Inhibited by c AMP e) acts upon both liver and muscle cells, similar to glucagon. 12) Glycogen catabolism is best described by which of the following statementsa) In the brain, it yields glucose for skeletal muscle consumption b) It requires a debranching enzyme in the erythrocytes c) It is not a major pathway in the brain d) It uses phosphorylase for glucose residue cleavage from the reducing end of glycogen in liver e) It is stimulated by Insulin in liver. 13) During the breakdown of glycogen, free glucose is formed from which of the following ? a) Glucose residues in -1,4 glycosidic linkages b) The reducing end c) The non reducing end d) Glucose residues in -1,6 glycosidic linkages e) Hydrolysis of glucose-1-P 14) Which of the following statements explains the synthesis of glycogen directly from DGlucose ? a) It does not use glucose-1-P b) It requires a debranching enzyme c) It occurs in erythrocytes. d) It requires UDP-Glucose e) It requires a glucosyl transferase 15)The degradation of glycogen normally produces which of the followings ?

a) More glucose than Glucose-1-P b) More Glucose-1-P than Glucose c) Equal amount of Glucose and Glucose-1-P d) Neither Glucose nor Glucose-1-P e) Only Glucose-I-P 16) Which of the following statements about liver phosphorylase kinase is most accurate ? a) It is present in the inactivated form when epinephrine is released b) It phosphorylates phosphorylase to make it inactive c) It is stimulated by c AMP mediated phosphorylation cascade d) It is not affected by ATP e) It is phosphorylated in response to Insulin. 17) A newborn is found to have fasting hypoglycemia. The nursery staff begins overnight feeds by nasogastric tube because they find that the child consistently has low blood glucose. A liver biopsy and molecular studies demonstrate an absence of Glycogen synthetase. The normal function of this enzyme is to do which of the followings ? a) Remove glucose residues one at a time from glycogen in liver b) Remove glucose residues one at a time from glycogen in muscles c) Transfer UDP glucose to the non reducing end of glycogen primer d) Hydrolyze -1,6 bonds of glycogen e) Function as a glucosyl 4:6 transferase. 18) A 3-year-old child presents to the pediatrician for failure to thrive. A workup including an ultrasound of his liver shows cirrhosis. A biopsy of liver demonstrates a deficiency of an enzyme involved in glycogen synthesis. Which of the following is the most likely glycogen storage disease ? a) Type I -Von Gierkes disease b)Type II Pompes disease

c) Type III- Coris disease d) Type IV- Andersens disease e) Type V- McArdle disease 19) A new-born is experiencing failure to thrive. On physical examination, organomegaly is appreciated due to accumulation of glycogen in the lysosomes of several organs, including the heart, muscle and liver. A diagnosis of Pompes disease is made. Which of the following biochemical deficits are seen in this disorder ? a) Glycogenin deficiency b) An -1,6 glycosidase deficiency c) A glucose-6-phosphatase deficiency d) A glycogen phosphorylase deficiency e) A lysosomal glucosidase deficiency 20) A second year medical student decides to do research in a nutrition laboratory that is studying the effect of caffeine on cellular metabolism. Caffeine inhibits cAMP phosphodiesterase. If caffeine is added to cells, which of the following enzymes is phosphorylated and inactivated in liver ? a) Phosphorylase kinase b) Calmodulin c) Phosphorylase d) Protein kinase A e) Glycogen synthase 21) A 28-year-old professional cyclist has been training for an opportunity to go for a long race. His coach strongly suggests the intake of carbohydrates after the work out to ensure a muscle glycogen storage that can endure the 28-day race. The activity of muscle glycogen synthase in resting muscles is increased by the action of which of the followings? a)Epinephrine b) Glucagon c) Insulin

d) Phosphorylation e) Fasting and starvation. 22) An infant was brought in to the emergency room after her parents witnessed her having seizures. The childs blood glucose was 28mmol/L. After a thorough workup, a GSD was suspected, and a muscle biopsy was significant for the accumulation of dextrin, a form of glycogen with branching limited to only a few glucose molecules. Which of the following GSD is most likely the cause of hypoglycemia and subsequent seizures ? a) Type I -Von Gierkes disease b)Type II Pompes disease c) Type III- Coris disease d) Type IV- Andersens disease e) Type V- McArdle disease

Answers- 1) c, 2)-b, 11)-b, 21)-c, 12)-c, 22)-c.

3)-b, 13)-d,

4)-a,

5)-c,

6)-a, 16)-c,

7)-c, 17)-c,

8)-c, 18)-d,

9)-d 19)-e,

10)-a 20)-e,

14)-d, 15)-b,

Posted in Metabolism - Carbohydrates, Multiple Choice Questions

Multiple choice Questions- Pentose phosphate pathway (Solved)

Published August 26, 2012 | By Dr. Namrata Chhabra 1) Glucose breakdown in certain mammalian and bacterial cells can occur by mechanisms other than classic glycolysis. In most of these, glucose 6-phosphate is oxidized to 6phosphogluconate, which is then further metabolized by: a) an aldolase-type split to form glyceric acid and glyceraldehyde 3-phosphate. b) an aldolase-type split to form glycolic acid and erythrose 4-phosphate. c) conversion to 1,6-bisphosphogluconate. d) decarboxylation to produce keto- and aldopentoses e) oxidation to a six-carbon dicarboxylic acid. 2) Which of the following enzymes acts in the pentose phosphate pathway? a) 6-phosphogluconate dehydrogenase b) Aldolase c) Glycogen phosphorylase d) Phosphofructokinase-1 e) Pyruvate kinase 3) The oxidation of 3 mol of glucose by the pentose phosphate pathway may result in the production of: a) 2 mol of pentose, 4 mol of NADPH, and 8 mol of CO2. b) 3 mol of pentose, 4 mol of NADPH, and 3 mol of CO2. c) 3 mol of pentose, 6 mol of NADPH, and 3 mol of CO2. d) 4 mol of pentose, 3 mol of NADPH, and 3 mol of CO2. e) 4 mol of pentose, 6 mol of NADPH, and 6 mol of CO2.

4) In a tissue that metabolizes glucose via the pentose phosphate pathway, C-1 of glucose would be expected to end up principally in: a) Carbon dioxide b) Glycogen c) Phosphoglycerate d) Pyruvate e) Ribulose 5-phosphate 5) The main function of the pentose phosphate pathway is to: a) give the cell an alternative pathway should glycolysis fail b) provide a mechanism for the utilization of the carbon skeletons of excess amino acids c) supply energy d) supply NADH e) supply Pentoses and NADPH 6) Which of the following statements about the pentose phosphate pathway is correct? a) It generates 38 mol of ATP per mole of glucose consumed. b) It generates 6 moles of CO2 for each mole of glucose consumed c) It is a reductive pathway; it consumes NADH. d) It is present in plants, but not in animals. e) It provides precursors for the synthesis of nucleotides. 7) The reactions of the pentose phosphate pathway using glucose-6 phosphate as the initial substrate are best described by which of the following statements ? a) They produce 2 moles of NADPH for each mole of CO2 released. b) They generate 2 moles of adenosine triphosphate per mole of glucose-6-phosphate metabolized to ribulose-5 phosphate. c) They occur in the matrix of mitochondria.

d) They are not required for the production of NADPH in the mature red blood cells e) They are required for the metabolism of glucose in the muscles. 8) In the pentose phosphate pathway, the major products are which of the followings ? a) Ribulose and ATP b) Ribulose and NADPH c) Ribose and NADH d) Ribose and NAD+ e) Xylulose and ATP 9) Which of the following statements about the oxidative section of the pentose phosphate pathway is correct? a) The pentose phosphate pathway generates NADH. b) The pentose phosphate pathway oxidizes NADPH to NADP+ c) The rate-limiting reaction of the pentose phosphate pathway is catalyzed by glucose-6phosphatase. d) The pathway supplies ribose-5-phosphate and NADPH in the quantities the cells requires. e) The pathway is highly active in the fasting state. 10) Which of the following statements about the nonoxidative section of the pentose phosphate pathway is correct? a) The nonoxidative reactions of the pentose phosphate pathway are not reversible. b) Transketolase is an enzyme that transfers three-carbon units in the pentose phosphate pathway c) Transaldolase is an enzyme that transfers two-carbon units in the pentose phosphate pathway d) Pentoses can provide glycolytic intermediates in this phase of HMP pathway e) Pentoses can be metabolized to provide NADPH in this phase of HMP pathway. 11) Which of the following statements about the use of the NADPH generated from the pentose phosphate pathway is not correct?

a) NADPH generated from the pentose phosphate pathway is used for steroid synthesis. b) NADPH generated from the pentose phosphate pathway is used for the regeneration of glutathione to its reduced state. c) NADPH generated from the pentose phosphate pathway is used for the synthesis of fatty acids d) NADPH generated from the pentose phosphate pathway can be oxidized in the electron transport chain to provide 38 ATPs e) NADPH generated from the pentose phosphate pathway is used for the Macrophageal functions. 12) A 19- year-old, African-American male military recruit is about to be sent to Iraq on his assignment. In preparation for his tour of duty, he is given a prophylactic dose of primaquine to prevent malaria. Several days after he develops fatigue and hemolytic anemia. Which of the following enzymes is likely deficient ? a) Fructokinase b) Aldolase B c) Glucose-6-P dehydrogenase d) Galactokinase e) Galactosyl Transferase. 13) A 50 -year-old alcoholic male presents with pain, numbness, tingling and weakness in his feet. He is diagnosed with thiamine deficiency. Thiamine and ATP condense together to form thiamine pyrophosphate, a cofactor important for the enzymes that catalyze oxidative decarboxylation of (-keto acids) in the TCA cycle and also at one of the steps in pentose phosphate pathway, which out of the following enzymes requires TPP as a coenzyme ? a) Glucose-6-P dehydrogenase b) Transaldolase c) Transketolase d) 6-P-Gluconate dehydrogenase e) Gluconolactone hydrolase. 14) The key regulatory enzyme of HMP pathway is-

a) Glucose-6-P dehydrogenase b) Transaldolase c) Transketolase d) 6-P-Gluconate dehydrogenase e) Gluconolactone hydrolase. 15) Xylulose 5-phosphate can be formed from Ribulose 5-phosphate by the action ofa) Glucose-6-P dehydrogenase b) Transaldolase c) Transketolase d) Phosphopentose epimerase e)Phosphopentose isomerase. 16)-The pentose phosphate pathway is active in liver, adipose tissue, adrenal cortex, thyroid, erythrocytes, testis, and lactating mammary gland. Why is the pathway less active in skeletal muscle? a) Muscle tissue contains very small amount of dehydrogenases b) Muscle tissues do not require NADPH c) Pentoses the major product of this pathway are not required by muscle tissue d) Muscle tissue contains very small amount of enzymes of non oxidative phase e) All of the above. 17) In rapidly dividing cancer cells the need for large amount of ribose is fulfilled bya) Highly active Glucose-6-P dehydrogenase b) Highly active Transaldolase c) Highly active Transketolase d) Highly active 6-P-Gluconate dehydrogenase

e) Reversal of non oxidative phase using glycolytic intermediates.

Answers-

1)-d 6)-b 11)-d 16)-a

2)-a 7)-a 12)-c 17)-e

3)-c 8)-b 13)-c

4)-a 9)-d 14)-a

5)-e 10)-d 15)-d

Posted in Metabolism - Carbohydrates, Multiple Choice Questions

Gluconeogenesis- Multiple Choice Questions (Solved)

Published August 22, 2012 | By Dr. Namrata Chhabra 1) A 4-month-old boy is being evaluated for seizures, psychomotor retardation, and hypotonia. Work up reveals elevated serum levels of lactate, alanine, pyruvate and keto acids. Based on the clinical presentation,pyruvate carboxylase activity is measured using fibroblasts from a skin biopsy and is found to be markedly decreased. This enzyme is normally used to directly synthesize which of the following molecules ? a) Pyruvate b) Oxaloacetate c) Malate d) Acetyl co A e) - Keto glutarate 2) A 34-year-old woman presents with central obesity, relatively thin extremities and purple stria on her abdomen. Further work up reveals an excessive serum cortisol level and a blood sugar level of 258 mg/dl. Which of the following is the most likely cause of her hyperglycemia ? a) Excessive consumption of processed carbohydrates b) A pancreatic adenoma secreting ACTH and glucagon c) Cortisol induced inhibition of insulin d) Glucorticoids cause induction of Phosphoenolpyruvate carboxy kinase gene

e) Increased gluconeogenesis due to increased provision of substrates through fatty acid oxidation. 3) A 56-year-old, obese man complains of polydipsia, polyuria and fatigue. A glucose tolerance test confirms the diagnosis of diabetes. He is placed on Metformin, which works by which of the following mechanisms ? a) Inhibiting hepatic gluconeogenesis b) Increasing glucagon level c) Increasing cellular responsiveness to circulating insulin d) Stimulating the release or preformed insulin e) Replacing the need for endogenous insulin 4) An enzyme used in both glycolysis and gluconeogenesis is: a) 3-phosphoglycerate kinase. b) Glucose 6-phosphatase c) Hexokinase d) Phosphofructokinase-1 e) Pyruvate kinase 5) Which one of the following statements about gluconeogenesis is false? a) For starting materials, it can use carbon skeletons derived from certain amino acids. b) It consists entirely of the reactions of glycolysis, operating in the reverse direction. c) It employs the enzyme glucose 6-phosphatase. d) It is one of the ways that mammals maintain normal blood glucose levels between meals. e) It requires metabolic energy (ATP or GTP). 6) All of the following enzymes involved in the flow of carbon from glucose to lactate (glycolysis) are also involved in the reversal of this flow (gluconeogenesis) except:

a) 3-phosphoglycerate kinase. b) Aldolase c) Enolase d) Phosphofructokinase-1 e) Phosphohexoseisomerase 7) In humans, gluconeogenesis: a) can result in the conversion of protein into blood glucose. b) helps to reduce blood glucose after a carbohydrate-rich meal. c) is activated by the hormone insulin d) is essential in the conversion of fatty acids to glucose. e) requires the enzyme hexokinase. 8) Which of the following substrates cannot contribute to net gluconeogenesis in mammalian liver? a) Alanine b) Glutamate c) Palmitate d) Pyruvate e) -Keto glutarate. 9) A 6-year-old boy begins playing soccer in a community league. After his first game of the year, he is brought to see his pediatrician because of his severe muscle cramps and blood in urine. He is subsequently found to be having deficiency of lactate dehydrogenase. This enzyme is important in which of the following conversions ? a) Pyruvate to Acetyl co A b) Pyruvate to Oxaloacetate c) Pyruvate to Alanine

d) Pyruvate to Phosphoenol pyruvate e) Pyruvate to lactate 10) A new-born infant is found to be having persistent hypoglycemia, despite decreased feeding intervals. The child is also irritable with a moderate degree of hepatomegaly. He is found to have a normal level of muscle fructose 1,6 bisphosphatase but decreased level of liver isoform. Which of the following statement is true of fructose 1,6 bisphosphatase? a) It is induced by Adenosine mono phosphate (AMP) b) It is induced by Insulin c) It is inhibited by Fructose 2,6 bisphosphate d) It is induced in the fed state e) It is inhibited during fasting. 11) A 14-year-old high school girl who is extremely conscious about her appearance has gone a full day fasting to fit in to a dress she intentionally brought a size smaller than her actual size for a dance party. Which of the following organs/tissues contributes to the glucose that is being synthesized through gluconeogenesis ? a) Spleen b) Red blood cells c) Skeletal muscle d) Liver e) Brain 12) An 8 -year-old boy presents with frequent episodes of weakness, accompanied by sweating and feelings of dizziness. Physical examination is remarkable for palpably enlarged liver and kidneys. Blood biochemistry reveals lactic acidemia. The patient is diagnosed with deficiency of glucose-6-phosphatase, which is normally only expressed ina) Liver and muscle b) Liver and brain c) Erythrocytes d) Liver and adipose tissues

e) Liver and kidney. 13) A 32-year-old body builder has decided to go on a diet consisting of egg whites to ensure only proteins for muscle growth. After a few weeks he experiences decreased energy and is found to be hypoglycemic. A nutritionist tells the patient that he most likely has the deficiency of vitamin Biotin. Which of the following enzymes is unable to catalyze its step in synthesizing glucose from pyruvate? a) Pyruvate carboxylase b) Phospho enol pyruvate carboxy kinase c) Glucose-6-phosphatase d) Fr 1,6 bisphosphatase e) Phosphoglycerate kinase. 14) In an individual at rest, who has fasted for 12 hours, which of the following occurs ? a) Gluconeogenesis is the major process by which blood glucose is maintained b) Liver glycogen stores are depleted c) Glycogen synthase is activated in liver d) Adenylate cyclase is inactivated in liver e) Phosphorylase, pyruvate kinase and glycogen synthase are phosphorylated in liver. 15) Which of the following is a common intermediate in the conversion of glycerol and lactate to glucose ? a) Pyruvate b) Oxaloacetate c) Malate d) Glucose-6-phosphate e) Phosphoenolpyruvate. 16) In the conversion of pyruvate to glucose during gluconeogenesis, a) Biotin is required

b) CO2 added in one reaction, appears in the final product c) Energy is used up in the form of GTP d) All the reactions take place in the cytosol e) PDH is the allosteric enzyme used. 17) Which of the following glycolytic enzymes is used in gluconeogenesis ? a) Glucokinase b) Phosphofructokinase-1 c) Aldolase B d) Phosphoglycerate kinase e) Pyruvate kinase. 18) The conversion of pyruvate to fructose 1,6 bisphosphate by the gluconeogenic pathway involves which of the followings ? a) Requires lactate dehydrogenase b) Requires hexokinase c) Produces 2 ATP and 2GTP d) Requires 4 ATP and 2 GTP e) Acetyl co A is not required to activate this pathway. 19) A high ratio of insulin to glucagon can: a) Promote gluconeogenesis b) Promote glycogenolysis c) Found in Type 1 Diabetes Mellitus d) Occurs during fasting state e) Promotes synthesis of Glycogen 20) Which of the followings is a precursor for gluconeogenesis ?

a) Glycogen b) Glycerol c) Glucagon d) Galactose e) Glucose-1- P.

Answers- 1)-b , 2)-d, 3)-a, 4)-a, 5)-b, 6)-d, 7)-a, 8)-c, 9)-e, 10)-c, 11)-d, 12)-e, 13)-a, 14)-e, 15)-d, 16)-a ,17)-d, 18)-d, 19)-e, 20)-b.

Posted in Metabolism - Carbohydrates, Multiple Choice Questions

Blood Glucose Homeostasis- Power Point PresentationStudents corner

Published August 20, 2012 | By Dr. Namrata Chhabra Posted in Power point presentation, Power Point Presentations, Students' corner

TCA Cycle- Multiple Choice Questions (Solved)

Published August 19, 2012 | By Dr. Namrata Chhabra 1) A 24-year-old woman presents with diarrhea, dysphagia, jaundice and white transverse lines on the fingernails (Mees lines). The patient is diagnosed with Arsenic poisoning,that inhibits which of the following enzymes of TCA cycle ? a) Citrate synthase b) Isocitrate dehydrogenase

c) -Keto glutarate dehydrogenase complex d) Succinate dehydrogenase e) Malate dehydrogenase 2) A biochemistry graduate student isolated all enzymes of TCA cycle to produce NADH, oxidation of which of the following substrates in the citric acid cycle is not coupled to the production of NADH ? a) Succinate b) Malate c) -Keto glutarate d) Isocitrate e) Pyruvate. 3) A 3-Year-old boy presents to the pediatric clinic with the symptoms of hypotonia, lactic acidosis, and seizures. After an extensive workup , he is diagnosed with pyruvate dehydrogenase complex deficiency. Which of the following cofactors is not required by this enzyme to convert pyruvate to Acetyl co A ? a) Thiamine b) Lipoic acid c) Pantothenic acid d) Niacin e) Ascorbic acid. 4) Which of the following vitamins is required for the synthesis of a cofactor required for the conversion of Succinate to Fumarate? a) Thiamine b) Lipoic acid c) Pantothenic acid d) Niacin

e) Riboflavin. 5) Pyruvate dehydrogenase complex deficiency is an autosomal recessive disorder and leads to anion gap metabolic acidosis. Which of the following accumulates to cause metabolic acidosis ? a) Beta hydroxy butyric acid b) Acetoacetic acid c) Fumaric acid d) Lactic acid e) Hydrochloric acid. 6) A 3 year-old child presents with a history of recurrent rash upon sun exposure and passage of purple colored urine. The child is diagnosed with Congenital Erythropoietic Porphyria, a disorder of pathway of haem biosynthesis. Which of the following intermediates of TCA cycle is used as a precursor for haem biosynthesis ? a) Succinyl co A b) Acetyl co A c) Succinate d) Malate e) Pyruvate 7) A 16 -year-old male comes to the clinic for a routine sports physical examination. His physical examination is unremarkable except for the skin findings of multiple pale masses which appear to be xanthomas. Xanthomas are generally associated with the underlying hypercholesterolemia. Which of the followings is utilized as a precursor for cholesterol biosynthesis ? a) Succinyl co A b) Acetyl co A c) Succinate d) Malate e) Pyruvate.

8) A 2-year-old child was brought to pediatric emergency with convulsions. The child was diagnosed with ammonia intoxication due to some urea cycle disorder. Reduced formation of GABA is considered to be the most important cause of convulsion due to depletion of glutamate from where it is produced by decarboxylation. Which of the following intermediates of TCA cycle is involved in the formation of Glutamate ? a) Succinate b) Malate c) -Keto glutarate d) Isocitrate e) Pyruvate. 9) In TCA cycle , GTP is produced at one step by substrate level phosphorylation and that is subsequently utilized for Gluconeogenesis. Which of the following enzymes is involved in this process of formation of GTP from GDP ? a) Succinate Thiokinase b) Succinate dehydrogenase c) Citrate synthase d) Isocitrate dehydrogenase e) Malate dehydrogenase 10) A 5-year-old child was rushed to pediatric emergency as he accidently consumed Fluoroacetate, a known inhibitor of TCA cycle. Which of the following enzymes is inhibited by fluoroacetate? a) Citrate synthase b) Aconitase c) Succinate dehydrogenase d) Isocitrate dehydrogenase e) Malate dehydrogenase. 11) Malonate is an inhibitor of-

a) Citrate synthase b) Aconitase c) Succinate dehydrogenase d) Isocitrate dehydrogenase e) Malate dehydrogenase. 12) A 56-year- old chronic alcoholic has been brought in a semiconscious state to the medical emergency. Blood biochemistry reveals hypoglycemia with blood glucose level of 45 mg/dl. Which of the following intermediates of TCA cycle can be directly converted to phosphoenolpyruvate to trigger the pathway of gluconeogenesis ? a) Succinate b) Malate c) -Keto glutarate d) Oxaloacetate e) Pyruvate. 13) A 78-year-old male is brought to emergency with Acute myocardial infarction. Blood biochemistry reveals lactic acidosis. How much energy yield (ATP) per mol of glucose is expected in such a condition? a) 32 b)2 c) 34 d) 36 d) 38 14) Which of the following allosteric modulators is not effective in influencing the rate of TCA cycle ? a) NADH b) FADH2

c) Ca++ d) ADP e) ATP 15) How many molecules of CO2 are produced per mole of Acetyl co A in TCA Cycle ? a) 2 b) 1 c) 3 d) 0 e) 4 16) The cytosolic isoform of which of the following TCA cycle enzymes requires NADP+ as a coenzyme, that can further be utilized in the reduced state for reductive biosynthesis? a) Citrate synthase b) Aconitase c) Succinate dehydrogenase d) Isocitrate dehydrogenase e) Malate dehydrogenase. 17) Which of the following intermediates of TCA cycle is depleted in Type 1 Diabetes mellitus to suppress TCA cycle and to channel excess Acetyl co A towards pathway of ketogenesis ? a) Succinate b) Malate c) -Keto glutarate d) Oxaloacetate e) Pyruvate.

18)- Which of the following enzymes is Thiamine dependent and essential for glucose oxidation in the brain? a) Pyruvate dehydrogenase complex b) Acetyl co A carboxylase c) Transaldolase d) Succinyl-co A Thiokinase e) Succinate dehydrogenase 19)-Anaplerotic reactions are those that result in replenishing intermediates in the TCA cycle. Which of the following enzymes catalyzes an anaplerotic reaction? a) Malate dehydrogenase b)Pyruvate carboxylase c) Pyruvate kinase d) Citrate synthase e) Succinyl-co A Thiokinase

20) -Which of the following intermediates of TCA cycle cannot be utilized for gluconeogenesis ? a) Succinate b) Malate c) -Keto glutarate d) Oxaloacetate e) Acetyl co A.

Answers-1)-c d 6)-a

2)-a

3)-e

4)-e

5)-

7)-b c 12)-d 13)-b 18)-a 19)-b

8)-c

9)-a

10)-b

11)-

14)-b

15)-a

16)-d

17)-

20)-e

Posted in Metabolism - Carbohydrates, Multiple Choice Questions

Glycolysis- Multiple Choice Questions(Solved)- Set-2

Published August 17, 2012 | By Dr. Namrata Chhabra 1). A patient has chronic obstructive pulmonary disease, enough oxygen is not reaching her tissues. All of the followings are expected excepta) The electron transport chain would be inhibited b) Glycolysis would be activated by a low ATP/ADP ratio c) More than normal amounts of ATP would be synthesized . d) Concentrations of NADH and pyruvate would be lower than normal e) More than normal amounts of lactate would be produced 2). A 45- year- old woman is diagnosed with breast cancer. The oncologist orders a positron emission tomography(PET) scan of the head to rule out metastasis. This imaging modality covalently links a radioactive isotope most commonly to Glucose to appreciate highly active areas in the body such as tumor. Which of the following traps the tracer in the cell ?

a) Insulin b) GLUT-1 c) GLUT-4 d) Glucokinase e) PFK-1 3) A 45-year-old obese man complains of having to get up frequently to urinate at nighttime. He has also noticed that he is constantly thirsty and hungry. The patient is diagnosed with type 2 diabetes mellitus. If this is a problem at the level of glucose transporters , which tissue would be most affected? a) Liver b) Red blood cells c) Muscle and adipose tissue d) Brain tissue e) Small intestine. 4) A 24-year woman is getting training for her first marathon. Her coach instructed her to keep a pace that allows her to stay below her anaerobic threshold. Under such conditions pyruvate does not accumulate as it is converted toa) Ethanol b) Lactic acid c) Acetyl co A d) Alanine e) Oxaloacetate. 5) A patient presents with dizziness, fatigue and tremors. A fingerstick test indicates a blood glucose level of 45 mg/dl (hypoglycemia). Glucagon is released under such condition . Which of the following allosteric modifier of PFK-1 would be most effective in switching off glycolysis ? a) Low level of citrate

b) Low level of Fr, 2,6 bisphosphate c) Low level of ATP d) Low level of NAD+ e) Low level of Glucose-6-P 6) A 30 -year-old woman is enduring her second trimester of pregnancy. The greater affinity fetal hemoglobin acquires oxygen released from the lesser affinity maternal hemoglobin due to the effect of an allosteric modifier, which isa) Fr-2,6 bisphosphate b) 2,3 bisphosphoglycerate c) Citrate d) Acetyl co A e) Fr-1,6 bisphosphoglycerate 7) A pediatric hematologist sees an 18 month-old patient with jaundice, splenomegaly and hemolytic anemia. Blood smear indicates red blood cells that are more rigid in appearance than normal. A diagnosis of pyruvate kinase deficiency is made. What products would be made in low amounts ? a) Acetyl co A b) Glucose c) Lactate d) ATP e) Alanine 8) In an embryo with a complete deficiency of pyruvate kinase, how many net moles of ATP would be generated in the conversion of one mole of glucose to one mole of pyruvate ? a) 0 b) 1 c) 2

d) 3 e) 4 9) Which out of the following enzymes cleaves a carbon-carbon bond in the pathway of glycolysis ? a) Aldolase b) Glyceraldehyde-3-P dehydrogenase c) Enolase d) Phosphoglycerate kinase e) Phosphoglycerate mutase 10) After a heavy meal, which out of the following allosteric activator would be most effective in increasing the rate of glycolysis ? a) ATP b) Citrate c) Acetyl co A d) Fr-2,6 bisphosphate e) 2,3 bisphosphoglycerate. 11) Compared with the resting state a vigorously contracting muscle showsa) An increased conversion of pyruvate to lactate b) Increased oxidation of pyruvate to CO2 c) A decreased concentration of AMP d) Decreased level of fr-2,6 bisphosphate e) Decreased ratio of NADH/NAD+ 12) A 24 -year-old man presented with symptoms of shortness of breath, weakness and fatigue. His Hemoglobin level was 7G/dl. Red blood cell isolated from the patient showed abnormally low-level of lactate. A deficiency of which of the following enzymes would be the most likely cause for patients anemia?

a) Phosphoglucose isomerase b) Phosphofructokinase c) Hexokinase d) Glucokinase e) Pyruvate kinase. 13) A mixture of sodium fluoride and potassium oxalate is added to sample collected for blood glucose estimation to prevent loss of glucose by anaerobic glycolysis. Fluoride is an inhibitor of glycolysis. Which of the following enzymes is inhibited by fluoride ? a) Hexokinase b) Phosphofructokinase c) Glyceraldehyde-3-P dehydrogenase d) Lactate dehydrogenase e) Enolase 14) Oxamate is a competitive inhibitor of which of the following enzymes ? a) Hexokinase b) Phosphofructokinase c) Glyceraldehyde-3-P dehydrogenase d) Lactate dehydrogenase e) Pyruvate dehydrogenase 15) Which out of the following enzymes of glycolysis is inhibited by Arsenate ? a) Hexokinase b) Phosphofructokinase c) Glyceraldehyde-3-P dehydrogenase d) Lactate dehydrogenase

e) Pyruvate dehydrogenase Answers1)-e 2)-b 3)-c 4)-c 5)-b 6)-b

7)-d

8)-a

9)-a

10)-d

11)-a

12)-e

13)-e

14)-d

15)-c

Posted in Metabolism - Carbohydrates, Multiple Choice Questions

Glycolysis- Multiple Choice Questions (Solved)-Set-1

Published August 16, 2012 | By Dr. Namrata Chhabra 1) Which of these enzyme reactions is not irreversible in glycolysis? a) Hexokinase b) Glucokinase c) 3-phosphoglycerate kinase d) Phosphofructokinase-1 e) Pyruvate kinase 2) Which out of the following statements is not true about aerobic glycolysis ? a) The rate limiting enzyme is phosphofructokinase-1 which converts fructose-6- phosphate to fructose-1,6-bisphosphate b) The pathway is inhibited allosterically by fructose-2,6-bisphosphate and AMP c) The pathway takes place in mitochondria of every cell d) The pathway produces Pyruvate and NADH e) The pathway begins with glucokinase or hexokinase 3) The first step in the glycolytic pathway in Liver -

a) Is catalyzed by glucokinase b) Uses ATP and glucose as substrates c) Produces glucose-6-phosphate and ADP d) Is an irreversible step e) All of the above. 4) Phosphoglycerate kinase functions in carbohydrate metabolism to produce ATP via: a) Oxidative phosphorylation. b) Substrate level phosphorylation. c) Oxidative decarboxylation. d) Phosphorolysis e) Oxidative deamination 5). The phosphofructokinase-2 enzyme that transfers a phosphate group to fructose-6phosphate in glycolysisa) Is active in the phosphorylated form b) Catalyzes a reversible reaction c) Produces fructose-2,6-bisphosphate as a product d) Is the rate limiting enzyme for glycolysis e) Produces ATP as a product. 6). The enzyme that produces NADH from a triose phosphate in the glycolytic pathwaya) Uses NAD+ and dihydroxyacetone phosphate as substrates b)Produces 3-phosphoglycerate and NADH c) Is reversible d) Is called 3-phosphoglycerate kinase e) Uses FADH2 and glyceraldehyde-3-phosphate as substrates

7). The enzyme that catalyzes first substrate level phosphorylation in glycolysisa) Produces 3-phosphoglycerate as a product b) Produces ADP from AMP c) Is called glyceraldehyde-3-phosphate dehydrogenase d) Is called phosphofructokinase e) Is the rate limiting enzyme of glycolysis. 8). The enzyme that catalyzes the second substrate level phosphorylation of glycolysisa) Is called phosphoglyceromutase b) Produces lactate as a product c) Uses phosphoenolpyruvate as a substrate d) Catalyzes a reversible reaction e) Is stimulated by Glucagon. 9). Which of the following statements about the Lactate dehydrogenase (LDH) reaction is false a) The enzyme converts pyruvate to lactate b)The enzyme converts NADH to NAD+ c) The reaction is reversible d) It is the last enzyme reaction in glycolysis e) The enzyme is highly active under aerobic conditions 10). The two major factors determining whether a cell oxidizes glucose by aerobic glycolysis or by anaerobic glycolysis area) FADH2 and the number of mitochondria b) NADH and the ATP/ADP ratio c) Ca++ and AMP

d) Oxygen pressure and the number of mitochondria e) Presence of low Glucose and high AMP 11). When glucose is converted to lactate by anaerobic glycolysis, the equivalent of ________ ATPs are derived. When glucose is completely oxidized to CO2 by glycolysis, Pyruvate dehydrogenase, and the TCA cycle, the equivalent of _______ ATPs are derived. a) 7; 20 b) 2; 38 c) 7; 32 d)2; 12 e) 7; 25 12).Choose the incorrect statement about the condition When one molecule of glucose is oxidized to 2 molecules of lactate during anaerobic glycolysisa) One molecule of ATP is used by the phosphofructokinase-1 reaction b)One molecule of ATP is used by either the glucokinase or hexokinase reaction c) Two molecules of ATP are produced by the Glyceraldehyde -3-P dehydrogenase reaction d) Two molecules of ATP are produced by the pyruvate kinase reaction e) No ATP is produced at the level of lactate dehydrogenase reaction. 13). All of the following help to explain some cases of Lactic Acidosis excepta) High altitude b) Heart failure c) Not enough oxygen to satisfy the needs of oxidative phosphorylation d) Congenital deficiency of liver lactate dehydrogenase e) Inhibition of the electron transport chain 14) . All of the following are part of the Cori Cycle excepta) Lactate is the result of anaerobic glycolysis in a number of tissues

b) Lactate is transported to the liver c) Lactate is converted to glucose in the liver d) ATP is produced by the conversion of lactate to glucose e) Glucose is transported from liver to other tissues 15). The ATP/AMP ratio has a major effect upon the rate of ATP production by glycolysis. ATP and AMP bind to allosteric sites ona) Hexokinase b) Glucokinase c) Phosphofructokinase-1 d) Phosphofructokinase-2 e) 3-phosphoglycerate kinase 16). Which of the following statements about pyruvate kinase is false a) Can convert Phosphoenol pyruvate to pyruvate b) Is regulated by feed forward regulation c) Is active in the dephosphorylated form d) Is also involved in gluconeogenesis e) Deficiency causes haemolytic anemia. 17). Pasteur effect is due to inhibition of glycolysis by high concentration of a) Creatine phosphate b) ATP c) ADP d) AMP e) Pi

18). In the liver, glucagon causes a decrease in the glycolytic pathway by all of the following mechanisms excepta) Increased binding of GTP to G-protein b) Activation of the cAMP cascade c) Increased phosphorylation of enzymes by protein kinase A d) Activation of fructose-2,6-bisphosphatase and inhibition of phosphofructokinase-2 e) Increased binding of fructose-2,6-bisphosphate to phosphofructokinase-1 19). An increased concentration of insulin after meals causes an increase in the rate of glycolytic pathway by all of the following mechanisms excepta) Increase uptake of glucose in adipose tissue and muscle b) Inactivation of the cAMP cascade c) Increased dephosphorylation of enzymes activated by protein kinase A d) Activation of fructose-2,6-bisphosphatase and inhibition of phosphofructokinase-2 e) Induction of Glucokinase and PFK-1 20).During fasting state, the insulin to glucagon ratio drops and all of the following occur excepta) Protein kinase A will phosphorylate pyruvate kinase b) The pyruvate kinase step will be activated c) Protein kinase A will phosphorylate phosphofructokinase-2/fructose-2,6-bisphosphatase d) The concentration of fructose-2,6-bisphosphate will decrease e)The liver will not be able to synthesize pyruvate from phosphoenolpyruvate. 21) A high concentration of Glucose-6-P is inhibitory to which of the following enzymes ? a) Hexokinase b) Glucokinase c) PFK-1

d) PFK-2 e) All of the above,

Answers-

1)-c,

2)- b,

c)-e,

4)-b,

5)-c,

6)-b,

b,

7)-a, 12)-c

8)- c,

9)-e,

10)-b,

11)-

13)-d

14)-d

15)-c

16)-d

17)-b

18)-e

19)-d

20)-b

21)-a

Posted in Metabolism - Carbohydrates, Multiple Choice Questions