Metabolism of Carbohydrates

Difficulty Level : One

QNo: 1 QStem: The heptose ketose sugar formed in HMP shunt is : Ch1: Ch2: Ch3: Ch4: Reference: Explanation: Glucoheptose Sedoheptose Sedoheptulose Mannoheptose Harper's Biochemsitry 24th Edition. PP : 207 The answer is 'C' :

A molecule of Sedoheptulose-7-P and one of Glyceraldehyde-3-P are formed by the Transketolase reaction in HMP shunt.

Transketolase (with the help of coenzyme thiamine pyrophosphate, TPP) transfers a two-carbon unit comprising carbon 1 and 2 of a ketose to the aldehyde carbon of an aldose sugar. Therefore, it converts an Aldose (Ribose-5-P) to a ketose with two carbon more (Sedoheptulose-7-P).

QNo:

2 QStem: Glucose yields how many ATPs by Glycolysis (1 NADH = 3 ATP)? Ch1: Ch2: Ch3: Ch4: Reference: Explanation: 5 11 8 10

Chawla and Tarek, PP: 230 The answer is 'C' :

Oxidation of Glucose by Glycolysis yields : y One NADH at Gl-3-P DH step. y Two ATPs are generated by substrate level phosphorylation at phosphoglycerokinase & Pyruvate kinase steps. y Since one glucose gives rise to two Gl-3-P, net yield is 2 NADH and 4 ATP. y One NADH can roughly be considered to yield 3 ATPs, therefore 2 NADH = 6 ATP. Total = 10 ATP. Two ATPs are utilized for phosphorylation of glu and fru-6-P. Therefore net yield is 10 - 2 = 8 ATP QNo: 3 QStem: Glycolysis of Glucose, under anaerobic conditions, yields how many ATPs? Ch1: Ch2: Ch3: Ch4: Reference: 2 6 8 10

Chawla and Tarek, PP: 230

Explanation: The answer is 'A' : Under Anaerobic conditions, NADH cannot yield ATP as Electron transport chain is blocked. Therefore, Pyruvate is converted to Lactate by using one ( x 2 ) NADH. Net yield = 2 ATP only.

QNo: 4 QStem: Which one of the following is an intermediate in HMP shunt Ch1: Ch2: Ch3: Ch4: D-Ribulose D-Arabinose D-Xylose D-Lyxose Harpers Biochemistry 26th Edition, pp: 164. The answer is 'A' :

Reference: Explanation:

Ribulose-5-P is the first pentose sugar of HMP shunt generated by oxidative decarboxylation of 6-Phosphogluconate with the generation of the second molecule of NADPH. The only potent distractant in the options is Xylose, but it is Xylulose-5-P that is formed from Ribulose and not Xylose-5-P.

QNo: Ch1: Ch2: Ch3: Ch4:

86 QTopic: Galactose-6-phosphate Galactose-1,6-diphosphate Galactose-1-phosphate All of the above Reference:

Metabolism of CHODifficulty Level:

QStem: Galactose is phosphorylated by galactokinase to form

Lehninger's Biochemistry. 5th Edition. PP : 536

Explanation:

The answer is 'C' :

D-Galactose, a product of hydrolysis of the disaccharide lactose (milk sugar), passes in the blood from the intestine to the liver, where it is first phosphorylated at C-1, at the expense of ATP, by the enzyme galactokinase: Galactose + ATP --> galactose 1-phosphate + ADP QNo: 79 QTopic: Metabolism of CHODifficulty Level: 2

QStem: The reaction involved in conversion of succinyl CoA to succinate in TCA cycle requires Ch1: Ch2: Ch3: CDP ADP GDP

Ch4:

NADP+ Reference: Biochemistry by Lubert Stryer. 3rd Edition. PP : 376 The answer is 'C' :

Explanation:

The enzyme Succinyl CoA synthetase mediates this substrate level phosphorylation reaction. The cleavage of the thioester bond of Succinyl CoA is coupled to the phosphorylation of GDP. QNo: 70 QTopic: Metabolism of CHODifficulty Level: 2 QStem: Hexokinase has a high affinity for glucose than Ch1: Ch2: Ch3: Ch4: Fructokinase Galactokinase Glucokinase All of the above Reference: Explanation: Biochemistry by Lubert Stryer, 3rd Edition. PP : 361 The answer is 'D' :

The students would be tempted to answer 'C' Glucokinase. But this is a distractor.

All the enzymes in the options have lesser affinity (if not zero) than that of hexokinase. QNo: 65QTopic: Metabolism of CHODifficulty Level: 2

QStem: Cyclic AMP is formed from ATP by the enzyme adenylate cyclase which is activated by the hormone Ch1: Ch2: Ch3: Ch4: Insulin Epinephrine Testosterone Progesterone Reference: Explanation: Chatterjea and Shinde, 5th Edition, 2005. PP : 289 The answer is 'B' :

Epinephrine and Glucagon stimulate adenylate cyclase on the plasma membrane and enhance the synthesis of cAMP. cAMP is the second messenger for these hormones and stimulates a

cascade of kinase enzymes that result in phosphorylation of a number of proteins. The phosphorylation / dephosphorylation is a major mechanism of QNo: Ch1: Ch2: Ch3: Ch4: 66QTopic: Thyroid hormones Epinepherine Prolactin Follicle Stimulating Hormone Reference: Explanation: . Metabolism of CHODifficulty Level: 2

QStem: The synthesis of adenylate cyclase is increased by :

The Answer is 'A' :

Thyroid hormones stimulate the synthesis of a number of proteins including adenylate cyclase. Epinephrine, although acts through stimulation of adenylate cyclase, does not increase its synthesis.

QNo: Ch1: Ch2: Ch3: Ch4:

15QTopic: Metabolism of CHO D-Ribulose D-Arabinose D-Xylose D-Lyxose Reference: . The answer is 'A' :

Difficulty Level:

QStem: Which one of the following is an intermediate in HMP shunt

Explanation:

Ribulose-5-P is the first pentose sugar of HMP shunt generated by oxidative decarboxylation of 6-Phosphogluconate with the generation of the second molecule of NADPH. The only potent distractant in the options is Xylose, but it is Xylulose-5-P that is formed from Ribulose and not Xylose-5-P.

QNo:

9 QTopic: Metabolism of CHO

Difficulty Level:

QStem: Which of the following can be said to act as a carrier of Citric acid cylce Ch1: Ch2: Ch3: Ch4: ACETYL CoA a-KETOGLUTARATE MALATE OXALOACETATE Reference: Explanation: . The answer is 'D' :

Oxaloacetic acid (OAA) can be called as the carrier of TCA cycle because Acetyl CoA enters the cycle after combining with OAA to from Citric acid. At the end of each cycle, OAA is regenerated to accept a new Acetyl CoA unit. The pace of TCA cycle is also dependent upon the availability and

QNo:

42QTopic:

Metabolism of CHODifficulty Level:

QStem: Which of the following glycolytic enzymes is inhibited by fluoride: Ch1: Ch2: Ch3: Ch4: Glyceraldehyde 3-P dehydrogenase Phospholglycerate kinase Enolase Fructose1-6 diphosphate Reference: Explanation: Chatterjea and Shinde, Sixth Edition PP : 272 The answer is 'C' :

Fluoride inhibits ENOLASE enzyme that carries out the formation of Phosphoenol pyruvate from 2-Phosphoglycerate during glycolysis. Fluoride is added to the vials for collection of blood samples for estimation of blood glucose. Fluoride inhibits Enolase and hence stops the utilisation of glucose by RBCs in the blood samples. In the absence of fluoride glucose estimation could give false low values.

QNo: Ch1: Ch2: Ch3: Ch4:

43QTopic: Glucose-6-phosphatase Debranching enzyme Acid maltase Liver phosphorylase Reference:

Metabolism of CHODifficulty Level:

QStem: Pompe's disease is characterized by the deficiencey of :

Chatterjea and Shinde, Sixth Edition PP : 294

Explanation:

The answer is 'C' :

Pompe's disease is caused by the inherited deficiency of Acid maltase, lysosomal enzyme that hydrolyses oligosaccharides. Inheritence : Autosomal recessive.

'Normal' type of glycogen is deposited in excess in heart, liver, smooth and striated muscles. Cardiomegali and muscle hypotonia are the features and cardiac failure is the cause of death.

QNo:

45QTopic:

Metabolism of CHODifficulty Level:

QStem: All of the following antibodies are Beta-cytolytic (Pancreatic beta cells), except: Ch1: Ch2: Ch3: Ch4: Insulin Ab Islet cell Ab Glutamic acid decarboxylase Ab Insulin receptor Ab Reference: Explanation: . The answer is 'D' :

Type II diabetes mellitus is now recognised as an autosomal disease. A number of antibodies have been implicated in the progressive destruction of beta cells leading to DM. Insulin antibodies which attach the insulin granules and deplete the beta cells, Islet cell antibodies attach the membrane antigens of islet cells.

Similarly Glutamic acid decarboxylase antobodies that attach the lysosomal enzyme are also cytolytic and are being used as markers for developing DM. Insulin receptor antibodies bind with the Insulin receptors in peripheral tissues and make them unavailable to Insulin leading to insulin resistance. These antibodies donot bind to the beta cells.

QNo:

46QTopic:

Metabolism of CHODifficulty Level:

QStem: Which of the following enzyme carries out an anapleurotic reaction: Ch1: Ch2: Ch3: Ch4: Pyruvate kinase Pyruvate carboxylase Pyruvate carboxykinase Pyruvate dehydrogenase Reference: Explanation: Lehninger's Biochemistry, 5th Edition. PP : 617 The answer is 'B' :

Anapleurotic reactions or 'Filling-up' reactions are those that provide intermediates of the TCA cycle and hence increase the pace of the cycle.

The major enzymes that carryout anapleurotic reactions are : a) Malic enzyme - produces Malate from Pyruuvate b) Pyruvate carboxylase - produces Oxaloacetate from Pyruvate

c) Transaminases that pour amino acid carbon skeleton into the cycle.

QNo:

50QTopic:

Metabolism of CHODifficulty Level:

QStem: Lumber spinal fluid glucose concentration is characteristically low in patients with: Ch1: Ch2: Ch3: Ch4: Meningioma Cerebral haemorrhage Multiple sclerosis Coccal Meningitis Reference: Chatterjea and Shinde, Sixth Edition PP : 639

Explanation:

The answer is 'D' :

Bacterial infection in the CSF lowers the glucose concentration as the bacterial utilise the glucose.

All the other options in the question are non-infectious disease conditions.

QNo:

61QTopic:

Metabolism of CHODifficulty Level:

QStem: The GIT absorption of glucose is interferred by the deficiency of: Ch1: Ch2: Ch3: Ch4: Vitamin A Thiamine Biotin Insulin Reference: Explanation: Chatterjea and Shinde, 5th Edition, 2005. PP : 264 The answer is 'B' :

The absorption of glucose from GIT is diminished in case of deficiency of a number of B-complex vitamins e.g. Thiamine, Pyridoxine and pentothenic acid.

QNo:

62QTopic:

Metabolism of CHODifficulty Level:

QStem: Glucose absorption from intestine may be decreased in case of severe Ch1: Ch2: Ch3: Ch4: Oedema Nephritis Rickets Osteomyelitis Reference: Explanation: Chatterjea and Shinde, 5th Edition, 2005. PP : 264

The answer is 'A' :

The transporters responsible for absorption of glucose need sufficient concentration of Sodium for the abswortion of glucose since Sodium is Cotransported along with Glucose.

Oedema, most often than not, is associated with low sodium concentrations therefore low sodium availability can affect the glucose absorption.

QNo: Ch1: Ch2: Ch3: Ch4:

63QTopic: Glucose Insulin Cyclic AMP Citrate Reference:

Metabolism of CHODifficulty Level:

QStem: Glycogen synthetase activity is depressed by :

Chatterjea and Shinde, 5th Edition, 2005. PP : 293

Explanation:

The answer is 'C' :

Epinephrine and glucagon hormones act through the formation of cAMP. The mechanism involves a cascade of phosphorylation reactions mediated by a number of kinases. Glycogen Synthase is phosphorylated by the cAMP stimulated Protein Kinase. The phosphorylated Synthase in inactive.

QNo:

64QTopic:

Metabolism of CHODifficulty Level:

QStem: The branching enzyme acts on the glycogen when the glycogen chain has been lengthened to a minimum of _____ glucose units: Ch1: Ch2: Ch3: Ch4: 5 8 11 16 Reference: Explanation: Lehininger's Biochemistry, 5th Edition. PP : 568 The answer is 'C' :

Glycogen synthase cannot make the (a1-6) bonds found at the branch points of glycogen; these are formed by the glycogen-branching enzyme, also called amylo (1-4) to (1-6) transglycosylase or glycosyl-(4-6)-transferase. The glycogen-branching enzyme catalyzes transfer of a terminal fragment of 6 or 7 glucose residues from the nonreducing end of a glycogen branch having at least 11 residues to the C-6 hydroxyl group of a glucose residue at a more interior position of

the same or another glycogen chain, thus creating a new branch.

QNo:

67QTopic: enzyme Ch1: Ch2: Ch3: Ch4:

Metabolism of CHO

Difficulty Level:

QStem: Which of the following is a substrate for Lactose synthase UDP-GLucose UDP-Galactose Galactose Galactose-1-Phosphate Reference: Explanation: Biochemistry by Lubert Stryer, 3rd Edition. PP : 340 The answer is 'B' :

Lactose synthase consists of tow subunits viz . a Catalytic subunit (galactosyl transferase) and a-lactalbumin. The catalytic subunit transfers Galactosyl residue from a UDP-Galactose to Glucose and forms a B-1-4 glycosidic linkage. Lactalbumin is the modifier subunit and serves to modify the specificity of the catalytic subunit so that Galactose is transferred to Glucose rather than N-acetylglucosamine (for the synthesis of glycoproteins).

QNo:

68QTopic:

Metabolism of CHODifficulty Level:

QStem: The coenzyme essential for activity of muscle Glycogen phosphorylase is Ch1: Ch2: Ch3: Ch4: Cyclic AMP NAD+ Pyridoxal phosphate Coenzyme A Reference: Explanation: Lehininger's Biochemistry, 5th Edition. PP : 562 The answer is 'C' :

Pyridoxal phosphate is an essential cofactor in the glycogen phosphorylase reaction; its phosphate group acts as a general acid catalyst, promoting attack by Pi on the glycosidic bond.

This is an unusual role for this cofactor; its more typical role is as a cofactor in transamination and decarboxylation reactions.

QNo:

69QTopic:

Metabolism of CHODifficulty Level:

QStem: Inactive muscle phosphorylase b is activated by active Phosphorylase kinase which is stimulated by : Ch1: Ch2: Ch3: Ch4: Calcium ions Cyclic AMP Cyclic GMP A&B Reference: Explanation: Biochemistry by Lubert Stryer, 3rd Edition. PP : 462 The answer is 'D' :

The muscle glycogenolysis responds not only to the hormonal regulation but is also synchronised with muscle contraction. The hormonal arm works through cAMP whereas the synchronisation with muscle contraction is provided by Calcium ions. The delta subunit of Phosphorylase kinase is Calmodulin, a calcium binding protein that regulates many enzymes in eukaryotes.

QNo:

71 QTopic:

Metabolism of CHO

Difficulty Level:

QStem: Fructose - 1,6 -diphosphate is converted to Fructose-6phosphate by the enzyme Fructose-1.6-bisphosphatase which is stimulated by Ch1: Ch2: Ch3: Ch4: Glucagon Insulin AMP None of the above Reference: Explanation: Biochemistry by Lubert Stryer, 3rd Edition. PP : 443

The answer is 'A' :

Interconversion of Fr-6-P and Fr-1,6-BP is a key point of control. AMP stimulates Phosphofructokinase (PFK), whereas it inhibits the enzyme Fr1,6bisphosphatase (FBPase). Citrate has the opposite effect.

During starvation (high Glucagon), the level of Fr-2,6-BP drops due to phosphoryltic inhibition of Phsophofructokinase2. Low FBP2 decreases the activity of PFK and increases that of FBPase. Consequently Fr-1,6-BP is converted to Fr-6-P to generate glucose by gluconeogenesis.

QNo:

72 QTopic:

Metabolism of CHODifficulty Level:

QStem: Phosphofructokinase 2 (PFK-2) is a 'tandem enzyme', which holds another enzyme activity on it, which is : Ch1: Ch2: Ch3: Ch4: Phosphofructokinase 1 Fructose-bisphosphatase 2 Aldolase Triose-phosphate isomerase Reference: Explanation: Biochemistry by Lubert Stryer, 3rd Edition. PP : 365 The answer is 'B' :

PFK2 mediates the synthesis of Fru-2,6-bisphosphate (FBP2), which is major regulator of glycolysis and gluconeogenesis.

The enzyme that hydrolysis FBP2 is Fr-2,6-bisphosphatase. The two enzymes activities are present in a single 53 kD polypeptide chain, which thus is called a tandem enzyme. The activites of FBP2 and FBP2ase are reciproclly regulated by phosphorylation of a single serine residue. When glucose is scarce, Glucagon triggers this phosphorylation activating the FBPase and inhibits PFK2.

QNo: Ch1: Ch2: Ch3: Ch4:

73 QTopic: Glycerol-3-Phosphate Dihydroxyacetone phosphate 1,3-Bisphosphoglycerate All of the above Reference:

Metabolism of CHODifficulty Level:

QStem: In liver, Glyceraldehyde-3-phosphate may be converted to

Lehninger's Biochemistry, 3rd Edition. PP : 804

Explanation:

The answer is 'D' :

Glyceraldehyde-3-P has multiple fates in Liver - it can be converted to Glycerol-3-P for the sythesis of triglycerides, or to DHAP for glycolysis or to 1,3-bisphosphoglycerate by Rappaport Lubering shunt.

QNo: Ch1: Ch2: Ch3: Ch4:

74 QTopic: QStem: In the presence of Arsenate

Metabolism of CHODifficulty Level:

Enzyme Glyceraldehyde3-P dehydrogenase is inhibited Enzyme Phosphoglycerokinase is inhibited Substrate level phosphorylation is uncoupled from glycolysis 1,3-di-arsenoglycerate is produced Reference: Chatterjea and Shinde, 5th Edition, 2005. PP : 271 The best answer is 'A' :

Explanation:

Although the staement is not exactly true. The enzyme Glyceraldehyde-3-P DH carries out the conversion of Glyal-3-P to 1,3-Diphosphoglycerate. Arsenate competes with inroganic phosphate in the reaction and produces 1-Arseno-3-phsopho-glyceratewhich hydrolyses spontaneously to yield 3phosphoglycerate and heat. Therefore, the enzyme is not inhibited but is not able to form its normal product.

QNo:

75 QTopic:

Metabolism of CHODifficulty Level:

QStem: Coenzyme for the reoxidation of reduced lipoate in pyruvate dehydrogenase reaction is Ch1: Ch2: Ch3: Ch4: FAD+ NADP+ NAD+ FMN Reference: Explanation: Chatterjea and Shinde, 5th Edition, 2005. PP : 277 The answer is 'A' :

Enzyme Dihydrolipoyl dehydrogenase, involved in the reoxidation of lipoic acid, is a flavoprotein and contains FAD as the prosthetic group. The hydrogen from reduced DH-lipoic acid are recieved by FAD to form FADH2 which transfers them onward to NAD. Therefore, although the final recipient in the reaction is NAD, the enzyme first accepts the hydrogens on FAD molecule.

QNo:

76 QTopic:

Metabolism of CHODifficulty Level:

QStem: The approximate number of copies of dihydrolipoyl transacetylase in E.Coli Pyruvate dehydrogenase complex is : Ch1: Ch2: Ch3: Ch4: 8 24 34 1 Reference: Explanation: Lehninger's Biochemistry, 3rd Edition. PP : 604 The answer is 'B' :

The PDH complex contains three enzymespyruvate dehydrogenase (E1), dihydrolipoyl transacetylase (E2), and dihydrolipoyl dehydrogenase (E3)each present in multiple copies. The number of copies of each enzyme and therefore the size of the complex varies among species. In the bovine enzyme, 60 identical copies of E2 form a pentagonal dodecahedron (the core) with a diameter of about 25 nm. (The core of the Escherichia coli enzyme contains 24 copies of E2.)

QNo:

77 QTopic:

Metabolism of CHODifficulty Level:

QStem: Pyruvate is accumulated in case of the dietary deficiency of vitamin Ch1: Ch2: Ch3: Ch4: 383. Explanation: The answer is 'D' : Pyridoxine Folic acid Cobalamine Thiamine Reference: Biochemistry by Lubert Stryer. 3rd Edition. PP :

The major enzymes that has Thiamine pyrophosphate as coenzyme are : Pyruvate dehydrogenase, a-Keto-dehydrogenase and Transketolase.

Dietary deficiency or chronic alcoholism (excessive consumptoin of TPP) can affect the activity of these enzymes. Lower activity of Pyruvate dehydrogenase would lead to accumulation of pyruvate.

QNo: Ch1: Ch2: Ch3: Ch4:

78 QTopic: Ca++ Fe++ Zn++ Mg++ Reference:

Metabolism of CHODifficulty Level:

QStem: Citrate is converted to isocitrate by aconitase which contains:

Biochemistry by Lubert Stryer. 3rd Edition. PP : 375

Explanation:

The answer is 'B' :

Citrate is converted to Isocitrate by a dehydration followed by hydration steps. The enzyme Aconitase has been named after the intermediate 'Aconitate' in the reaction. The enzyme contains four Iron atoms complexed to four inorganic sulfides and four cysteine sulfur atoms. This Fe-S cluster binds Citrate and participates in dehydrating tha rehydrating the bound substrate. The enzyme Aconitase is thus an Iron-Sulfur protein.

QNo:

81 QTopic: tissues Ch1: Ch2: Ch3: Ch4:

Metabolism of CHO

Difficulty Level:

QStem: Glucose-6-phosphatase is absent from which of the following Brain Intestine Kidney Heart Reference: Explanation: Biochemistry by Lubert Stryer. 3rd Edition. PP : 454 The answer is 'A' :

The enzyme Glu-6-phosphatase is located on the luminal side of the smooth endoplasmic reticulum in the Liver cells and is essential for gluconeogenesis. The enzyme is also present in kidney and intestine but is absent in the brain tissue and muscles.

Brain and Muscle need a large amount of Glucose. The absence of Glu-6phosphatase helps them to retain glucose since Glu-6-P cannot escape the

QNo:

82 QTopic:

Metabolism of CHODifficulty Level:

QStem: 6-Phosphogluconate is converted to Ribulose-5-P in the presence of coenzyme Ch1: Ch2: Ch3: Ch4: FAD+ NAD+ NADP+ ATP Reference: Explanation: Lehninger's Biochemistry. 5th Edition. PP : 550 The answer is 'C' :

6-phosphogluconate undergoes oxidation and decarboxylation by 6-phosphogluconate dehydrogenase enzyme to form the ketopentose ribulose 5-phosphate.

The oxidation takes place in the presence of NAPD+ and requires Mg++ ions.

This reaction provides the second molecule of NADPH generated in HMP shunt.

QNo:

83 QTopic:

Metabolism of CHODifficulty Level:

QStem: UDP-Glucose is oxidized to UDP-glucuronic acid by a dehydrogenase in the presence of Ch1: Ch2: Ch3: Ch4: FAD+ NAD+ NADP+ ADP Reference: Explanation: . The answer is 'B' :

The uronic acid pathway is primarily involved in the synthesis of Glucuronic acid required for a) synthesis of glycosaminoglycans b) Glucuronides. UDP-G Dehydrogenase oxidises the carbon C6 of UDPG to form UDP glucuronic acid. The acceptor of hydrogen in the reaction is NAD+.

QNo: of CHO Ch1: Ch2: Ch3: Ch4:

84 Difficulty Level: Liver Adipose tissue Heart Brain Reference:

QTopic: 3

Metabolism

QStem: Fructokinase is present in which of the tissues

Lehninger's Biochemistry. 5th Edition. PP : 536

Explanation:

The answer is 'A' :

The pathway of entry of Fructose in the glycolytic pathway is different in the peripheral tissues and Liver. In the peripheral tissues, Fructose is phosphorylated by Hexokinase t Fru-6P. In liver, fructokinase phosphorylates it to Fru-1-P. Fr-1-P then is acted upon by Fr-1-P Aldolase to split it into glyceraldehyde and dihydroxyacetone

QNo:

85 QTopic:

Metabolism of CHODifficulty Level:

QStem: Fructose-1-phosphate is splitted into glyceraldehyde and dihydroxyacetone phosphate by the enzyme which is also known as Ch1: Ch2: Ch3: Ch4: Fructose-Biphosphate Aldolase Aldolase A Enolase Fructolase Reference: Explanation: IUB Website. The answer is 'A' :

The enzyme commission of International Union of Biologists and Biochemists now lists the Aldolase-1 and Aldolase-2 together as single enzyme but the enzyme cleaving Fr-1-P is also listed as Fructose-

QNo:

87

QTopic:

Metabolism of CHO

Difficulty Level:

QStem: In galactosemic individuals deficient in uridyltransferase, UDP-Galactose may be formed from Ch1: Ch2: Ch3: Ch4: Glucose UDP-Glucose Galactose-1-Phosphate All of the above Reference: Explanation: Lehninger's Biochemistry. 5th Edition. PP : 537

The answer is 'B' :

Galactosaemia is an inherited disorder (incidence 1 :18000) characterised by impaired utilization of Galactose, hence its accumulation in blood. The major enzyme defect is Gal-1-P Uridyl transferase but the deficiency of Galactokinase and Epimerase are also known causes of Galactosaemia.

Even if the patients are kept on a Galactose free diet, the Galactose can be formed from UDP-Glucose by epimerization to meet the body requirements of galactose.