Description : Defective enzyme in Hunter’s syndrome is (A) α-L-iduronidase (B) Iduronate sulphatase (C) Arylsulphatase B (D) C-acetyl transferase
Last Answer : Answer : B
Description : Hunter’s syndrome results from absence of (A) Hexosaminidase A (B) Iduronate sulphatase (C) Neuraminidase (D) Arylsulphatase B
Description : In Hunter’s syndrome (A) There is progressive corneal opacity (B) Keratan sulphate is excreted in the urine (C) Enzyme defective is arylsulphatase B (D) Hearing loss is perceptive
Last Answer : Answer : D
Description : Farber’s disease is due to the deficiency of the enzyme: (A) α-Galactosidase (B) Ceramidase (C) β-Glucocerebrosidase (D) Arylsulphatase A.
Description : Metachromatic leukodystrophy is due to deficiency of enzyme: (A) α-Fucosidase (B) Arylsulphatase A (C) Ceramidase (D) Hexosaminidase A
Description : The enzyme regulating extramitochondrial fatty acid synthesis is (A) Thioesterase (B) Acetyl CoA carboxylase (C) Acyl transferase (D) Multi-enzyme complex
Description : In Hurler’s syndrome, urine shows the presence of (A) Keratan sulphate I (B) Chondroitin sulphate (C) Dermatan sulphate and heparan sulphate (D) Keratan sulphate II
Last Answer : Answer : C
Description : Which of the following suggests a diagnosis of Hurler's Syndrome rather than Hunter's Syndrome? 1) X-linked inheritance 2) Mental retardation 3) Skeletal abnormalities 4) Cloudy cornea 5) Cardiomyopathy
Last Answer : Answers-4 Hunter's Syndrome (MPS-2) is of X-linked inheritance. The cornea are clear. The skeletal involvement tends to be mild with no gibbous present, though scoliosis is ... and gibbous deformation of the spine is characteristic. There is the characteristic coarse facies with hepatosplenomegaly.
Description : In glycogenolysis, the enzyme which transfers a trisaccharide unit from one branch to the other exposing 1→ 6 branch point is (A) Phosphorylase (B) α-[1→ 4]→ α-[1→ 4]→ Glucan transferase (C) Amylo [1→ 6] glucosidase (D) Amylo[1→ 4]→ [1→ 6] transglucosidase
Description : In glycogenesis a branch point in the molecule is established by the enzyme (A) Amylo[1→ 4][1→ 6] transglucosidase (B) α [1→ 4] α [1→ 4] Glucan transferase (C) Amylo [1→ 6] glucosidase (D) Glycogen synthase
Last Answer : Answer : A
Description : An inborn error, maple syrup urine disease is due to deficiency of the enzyme: (A) Isovaleryl-CoAhydrogenase (B) Phenylalnine hydroxylase (C) Adenosyl transferase (D) α-Ketoacid decarboxylase
Description : α-Galactosidase enzyme is defective in (A) Tay-sach’s disease (B) Refsum’s disease (C) Sandhoff’s disease (D) Fabry’s disease
Description : Lesch-Nyhan syndrome, the sex linked recessive disorder is due to the lack of the enzyme: (A) Hypoxanthine-guanine phosphoribosyl transferse (B) Xanthine oxidase (C) Adenine phosphoribosyl transferase (D) Adenosine deaminase
Description : Wolman’s disease is due to deficiency of (A) Cholesteryl ester hydrolase (B) Hexosaminidase A (C) α-Fucosidase (D) Arylsulphatase A
Description : Following enzyme is involved in the synthesis of actylcholine (a) Choline acetyl transferase (b) Acetyl cholinesterase (c) Both (a) and (b) (d) None of the above
Last Answer : Ans: A
Description : The enzyme deficient in metachromatic leukodystrophy is (A) Arylsulphatase A (B) Hexosaminidase A (C) Ceramidase (D) Sphingomyelinase
Description : Insulin regulates fatty acid synthesis by (A) Dephosphorylating of acetyl CoA carboxylase (B) Activating phosphorylase (C) Inhibiting malonyl CoA formation (D) Controlling carnitine-Acyl CoA transferase activity
Description : A flavoprotein in pyruvate dehydrogenase complex is (A) Pyruvate dehydrogenase (B) Didrolipoyl acetyl transferase (C) Dihydrolipoyl dehydrogenase (D) None of these ENZYMES 161
Description : Conversion of pyruvate into acetyl CoA is catalysed by (A) Pyruvate dehydrogenase (B) Didrolipoyl acetyl transferase (C) Dihydrolipoyl dehydrogenase (D) All the 3 acting in concert
Description : Acetyl-CoA is the principal building block of fatty acids. It is produced within the mitochondria and does not diffuse readily into cytosol. The availability of acetyl CoA involves (A) Carnitine acyl transferase (B) Pyruvate dehydrogenase (C) Citrate lyase (D) Thiolase
Description : Acetyl CoA required for extra mitochondrial fatty acid synthesis is produced by (A) Pyruvate dehydrogenase complex (B) Citrate lyase (C) Thiolase (D) Carnitine-acyl transferase
Description : The α-amino group of the new amino acyl tRNA in the A site carries out a nucleophilic attack on the esterified carboxyl group of the peptidyl tRNA occupying the P site. This reaction is catalysed by (A) DNA polymerase (B) RNA polymerase (C) Peptidyl transferase (D) DNA ligase
Description : Complete absence of hypoxanthine guanine phospharibosyl transferase causes (A) Primary gout (B) Immunodeficiency (C) Uric acid stones (D) Lesh-Nyhan syndrome
Description : Bilirubin UDP-glucuronyl transferase is absent from liver in (A) Crigler-Najjar syndrome, type I (B) Gilbert’s disease (C) Crigler-Najjar syndrome, type II (D) Rotor’s syndrome
Description : Which of the following concerning the conjugation of bilirubin is correct? 1) is catalysed by a glucuronyl transferase 2) occurs in the Kupfer cells of the liver 3) is increased by valproate 4) is inhibited by rifampicin 5) is impaired in Dubin-Johnson syndrome
Last Answer : Answers-1 b - Hepatocytes. c - Enzyme inhibitor. d - Enzyme inducer. e - Conjugation is OK but excretion from the hepatocyte into the bile is impaired. (Gilbert's syndrome ... bilirubinaemia. Dubin-Johnson syndrome - bilirubin can't Depart from the hepatocyte - conjugated bilirubinaemia.)
Description : Excretion of conjugated bilirubin from liver cells into biliary canaliculi is defective in (A) Gilbert’s disease (B) Crigler-Najjar syndrome (C) Lucey-Driscoll syndrome (D) Rotor’s syndrome
Description : The active transport system for hepatic uptake of bilirubin is congenitally defective in (A) Gilbert’s disease (B) Crigler-Najjar syndrome (C) Rotor’s syndrome (D) Dubin-Johnson syndrome
Description : For each of the first 7-acetyl-CoA molecules formed by α-oxidation of palmitic acid, the yield of high energy phosphates is (A) 12 (B) 24 (C) 30 (D) 35
Description : The initial step of the citric acid cycle is (A) Conversion of pyruvate to acetyl-CoA (B) Condensation of acetyl-CoA with oxaloacetate (C) Conversion of citrate to isocitrate (D) Formation of α -ketoglutarate catalysed by isocitrate dehydrogenase
Description : In synthesis of Triglyceride from α-Glycero phosphate and acetyl CoA, the first intermediate formed is (A) β-diacyl glycerol (B) Acyl carnitine (C) Monoacyl glycerol(D) Phosphatidic acid
Description : Which one of the following compounds cannot give rise to the net synthesis of Glucose? (A) Lactate (B) Glycerol (C) α-ketoglutarate (D) Acetyl CoA
Last Answer : B
Description : Before pyruvic acid enters the TCA cycle it must be converted to (A) Acetyl CoA (B) Lactate (C) α-ketoglutarate (D) Citrate
Last Answer : A
Description : The most important mechanism by which gram negative bacilli acquire chloramphenicol resistance is (a) Decreased permeability into the bacterial cell (b) Acquisition of a plasmid encoded ... bacterial ribosome for chloramphenicol (d) Switching over from ribosomal to mitochondrial protein synthesis
Last Answer : Ans: B
Description : The most important mechanism by which gram negative bacilli acquire chloramphenicol resistance is: A. Decreased permeability into the bacterial cell B. Acquisition of a plasmid encoded ... the bacterial ribosome for chloramphenicol D. Switching over from ribosomal to mitochondrial protein synthesi
Last Answer : B. Acquisition of a plasmid encoded for chloramphenicol acetyl transferas
Description : The enzyme required for salvage of free purine bases is (A) Adenine phosphoribosyl transferase (B) Hypoxanthine guanine phosphoribosyl transferase (C) Both (A) and (B) (D) None of these
Description : Orotic aciduria type II reflects the deficiency of the enzyme: (A) Orotate phosphoribosyl transferase (B) Orotidylate decarboxylase (C) Dihydroorotase (D) Dihydroorotate dehydrogenase
Description : Methotrexate blocks the synthesis of thymidine monophosphate by inhibiting the activity of the enzyme: (A) Dihydrofolate reductase (B) Orotate phosphoribosyl transferase (C) Ribonucleotide reductase (D) Dihydroorotase
Description : An enzyme which acts as allosteric regulator and sensitive to both phosphate concentration and to the purine nucleotides is (A) PRPP synthetase (B) PRPP glutamyl midotransferase (C) HGPR Tase (D) Formyl transferase
Description : In the synthetic pathway of epinephrine, disulfiram (antabuse) inhibits the enzyme: (A) Tyrosine hydroxylase (B) Dopamine β-hydroxylase (C) DOPA decarboxylase (D) N-methyl transferase
Description : A deficiency of copper effects the formation of normal collagen by reducing the activity of which of the following enzyme? (A) Prolyl hydroxylase (B) Lysyl oxidase (C) Lysyl hydroxylase (D) Glucosyl transferase
Description : The enzyme hexokinase is a (A) Hydrolase (B) Oxidoreductase (C) Transferase (D) Ligase
Description : In the biosynthesis of cholesterol, the rate limiting enzyme is (A) Mevalonate kinase (B) HMG-CoA synthetase (C) HMG-CoA reductase (D) Cis-prenyl transferase
Description : Synthesis of phosphatidylinositol by transfer of inositol to CDP diacylglycerol is catalysed by the enzyme: (A) CTP phosphatidate cytidyl transferase (B) Phosphatidate phosphohydrolase (C) CDP-diacylglycerol inositol transferase (D) Choline kinase
Description : Synthesis of polyunsaturated fatty acids involves the enzyme systems: (A) Acyl transferase and hydratase (B) Desaturase and elongase (C) Ketoacyl-CoA reductase and hydratase (D) Dihydroxyacetone phosphate
Description : Galactose 1-phosphate is converted to uridine diphosphate galactose, the reaction is catalysed by the enzyme: (A) Glactokinase (B) Galactose 1-phosphate uridyl transferase (C) Uridine diphospho galactose 4-epimerase (D) UDP glucose pyrophosphorylase
Description : Glycogen is converted to glucose-1- phosphate by (A) UDPG transferase (B) Branching enzyme (C) Phosphorylase (D) Phosphatase
Last Answer : C
Description : The defective enzyme in histidinemia is (A) Histidine carboxylase (B) Histidine decarboxylase (C) Histidase (D) Histidine oxidase
Description : Which is the defective enzyme in von Gierkeís disease (glycogen storage disease type I)?
Last Answer : Glucose-6-phosphatase.
Description : Niemann-Pick disease results from deficiency of (A) Ceramidase (B) Sphingomyelinase (C) Arylsulphatase A (D) Hexosaminidase A
Description : Tay-Sachs disease results from inherited deficiency of (A) Arylsulphatase A (B) Hexosaminidase A (C) Sphingomyelinase (D) Ceramidase