Hyperammonaemia type I results from congenital absence of (A) Glutamate dehydrogenase (B) Carbamoyl phosphate synthetase (C) Ornithine transcarbamoylase (D) None of these

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Answer :  B

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Description : Transfer of the carbamoyl moiety of carbamoyl phosphate to ornithine is catalysed by a liver mitochondrial enzyme: (A) Carbamoyl phosphate synthetase (B) Ornithine transcarbamoylase (C) N-acetyl glutamate synthetase (D) N-acetyl glutamate hydrolase

Last Answer : Answer : B

Description : Congenital deficiency of ornithine transcarbamoylase causes (A) Hyperammonaemia type I (B) Hyperammonaemia type II (C) Hyperornithinaemia (D) Citrullinaemia

Last Answer : Answer : C

Description : Control of urea cycle involves the enzyme: (A) Carbamoyl phosphate synthetase (B) Ornithine transcarbamoylase (C) Argininosuccinase (D) Arginase

Last Answer : Answer : A

Description : All the enzymes required for de novo synthesis of pyrimidine nucleotides are cytosolic except (A) Carbamoyl phosphate synthetase (B) Aspartate transcarbamoylase (C) Dihydro-orotase (D) Dihydro-orotate dehydrogenase

Last Answer : Answer : D

Description : De novo synthesis of pyrimidine nucleotides is regulated by (A) Carbamoyl phosphate synthetase (B) Aspartate transcarbamoylase (C) Both (A) and (B) (D) None of these

Last Answer : Answer : C

Description : An enzyme common to de novo synthesis of pyrimidine nucleotides and urea is (A) Urease (B) Carbamoyl phosphate synthetase (C) Aspartate transcarbamoylase (D) Argininosuccinase

Last Answer : Answer : B

Description : The metabolite which sustains urea cycle is (A) Ornithine (B) Citrulline (C) Carbamoyl phosphate (D) n-acetyl glutamate

Last Answer : Answer : C

Description : Carbamoyl phosphate synthetase structure is marked by change in the presence of (A) N-Acetyl glutamate (B) N-Acetyl Aspartate (C) Neuraminic acid (D) Oxalate

Last Answer : Answer : A

Description : Orotic aciduria type I reflects the deficiency of enzymes: (A) Orotate phosphoribosyl transferase and orotidylate decarboxylase (B) Dihydroorotate dehydrogenase (C) Dihydroorotase (D) Carbamoyl phosphate synthetase

Last Answer : Answer : A

Description : An enzyme of pyrimidine nucleotides biosynthesis regulated at the genetic level by apparently coordinate repression and derepression is (A) Carbamoyl phosphate synthetase (B) Dihydroorotate dehydrogenase (C) Thymidine kinase (D) Deoxycytidine kinase

Last Answer : Answer : A

Description : Enzyme deficient in Hyperammonemia type II is (A) Glutamine synthetase (B) Glutaminase (C) Ornithine transcarbamoylase (D) Carbamoylphosphate synthetase

Last Answer : Answer : C

Description : Hyperargininemia, a defect in urea synthesis develops due to deficiency of the enzyme: (A) Ornithine transcarbamoylase (B) Argininosuccinase (C) Arginase (D) Argininosuccinate synthetase ENZYMES 157

Last Answer : Answer : B

Description : An enzyme which is excreted in urine is (A) Lactase dehydrogenase (B) Amylase (C) Ornithine transcarbamoylase (D) None of these

Last Answer : Answer : C

Description : An important finding in Hyperammonemia type II is (A) Increased serum gluatmine level (B) Enlarged liver (C) Mental retardation (D) Increased carbamoyl phosphate synthetase level

Last Answer : Answer : C

Description : L-glutamic acid is subjected to oxidative deaminition by (A) L-amino acid dehydrogenase (B) L-glutamate dehydrogenase (C) Glutaminase (D) Glutamine synthetase

Last Answer : Answer : B

Description : Cytosolic carbamoyl phosphate synthetase is activated by (A) Glutamine (B) PRPP (C) ATP (D) Aspartate

Last Answer : Answer : B

Description : Cytosolic carbamoyl phosphate synthetase is inhibited by (A) UTP (B) CTP (C) PRPP (D) TMP

Last Answer : Answer : A

Description : Cytosolic and mitochondrial carbamoyl phosphate synthetase have the following similarity: (A) Both use ammonia as a substance (B) Both provide carbamoyl phosphate for urea synthesis (C) Both require N-acetylglutamate as an activator (D) Both are allosteric enzymes

Last Answer : Answer : C

Description : The enzyme carbamoyl phosphate synthetase requires (A) Mg++ (B) Ca++ (C) Na+ (D) K+

Last Answer : Answer : A

Description : CTP synthetase forms CTP from (A) CDP and inorganic phosphate (B) CDP and ATP (C) UTP and glutamine (D) UTP and glutamate

Last Answer : Answer : C

Description : Increased urinary excretion of orotic acid can occur in deficiency of (A) Orotate phosphoribosyl transferase (B) OMP decarboxylase (C) Mitochondrial ornithine transcarbamoylase (D) Any of the above

Last Answer : Answer : D

Description : Two nitrogen of the pyrimidines ring are obtained from (A) Glutamine and Carbamoyl-p (B) Asparate and Carbamoyl-p (C) Glutamate and NH3 (D) Glutamine and NH3

Last Answer : Answer : B

Description : In urea synthesis, the amino acid functioning solely as an enzyme activator: (A) N-acetyl glutamate(B) Ornithine (C) Citrulline (D) Arginine

Last Answer : Answer : A

Description : An enzyme of pyrimidine nucleotide biosynthesis sensitive to allosteric regulation is (A) Aspartate transcarbamoylase (B) Dihydroorotase (C) Dihydroorotate dehydrogenase (D) Orotidylic acid decarboxylase

Last Answer : Answer : A

Description : Haem synthetase is congenitally deficient in (A) Congenital erythropoietic porphyria (B) Protoporphyria (C) Hereditary coproporphyria (D) Variegate porphyria

Last Answer : Answer : B

Description : Infant i le convu ls ions due to lesser formation of gamma amino butyric acid from glutamic acid is seen in the deficiency of (A) Glutamate-dehydrogenase (B) Pyridoxine (C) Folic acid (D) Thiamin

Last Answer : Answer : B

Description : Coenzymes derived from the vitamin shown below are required by which of the following enzymes? (A) Lactate dehydrogenase (B) Glutamate dehydrogenase (C) Pyruvate dehydrogenase (D) Malate dehydrogenase

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Description : Biotin is a coenzyme for (A) Pyruvate dehydrogenase (B) Pyruvate carboxylase (C) PEP carboxykinase (D) Glutamate pyruvate transminase

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Description : Allsoteric activator of glutamate dehydrogenase is (A) ATP (B) GTP (C) ADP and GDP (D) AMP and GMP

Last Answer : Answer : C

Description : Allosteric inhibitor of glutamate dehydrogenase is (A) ATP (B) ADP (C) AMP (D) GMP

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Description : Most of the ammonia released from L-αamino acids reflects the coupled action of transaminase and (A) L-glutamate dehydrogenase (B) L-amino acid oxidase (C) Histidase (D) Serine dehydratase

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Description : I-cell disease results from absence of the following from lysosomal enzymes: (A) Signal sequence (B) Mannose-6-phosphate (C) Sialic acid (D) A serine residue

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Description : A key substance in the committed step of pyrimidines biosynthesis is (A) Ribose-5-phosphate (B) Carbamoyl phosphate (C) ATP (D) Glutamine

Last Answer : Answer : B

Description : The two nitrogen of the pyrimidine ring are contributed by (A) Ammonia and glycine (B) Asparate and carbamoyl phosphate (C) Glutamine and ammonia (D) Aspartate and ammonia

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Description : Pyrimidine biosynthesis begins with the formation from glutamine, ATP and CO2, of (A) Carbamoyl aspartate (B) Orotate (C) Carbamoyl phosphate (D) Dihydroorotate

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Description : The highest energy level is present amongst the following in (A) 1, 3-Biphosphoglycerate (B) Creatine phosphate (C) Carbamoyl phosphate (D) Phosphoenol pyruvate

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Description : Which of the following contributes nitrogen atoms to both purine and pyrimidine rings? (A) Aspartate (B) Carbamoyl phosphate (C) CO2 (D) Glutamine

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Description : ATP is required in following reactions of urea cycle: (A) Synthesis of carbamoyl phosphate and citrulline (B) Synthesis of citrulline and argininosuccinate (C) Synthesis of argininosuccinate and arginine (D) Synthesis of carbamoyl phosphate and argininosuccinate

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Description : Carbamoyl phosphate required for urea synthesis is formed in (A) Cytosol (B) Mitochondria (C) Both (A) and (B) (D) None of these

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Description : GMP is an allosteric inhibitor of all the following except (A) PRPP synthetase (B) PRPP glutamyl amido synthetase (C) IMP dehydrogenase (D) Adenylosuccinate synthetase

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Description : In the pathway of de novo synthesis of purine nucleotides, all the following are allosteric enzymes except (A) PRPP glutamyl amido transferase (B) Adenylosuccinate synthetase (C) IMP dehydrogenase (D) Adenylosuccinase

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Description : In the biosynthesis of purine nucleotides the AMP feed back regulates (A) Adenylosuccinase (B) Adenylosuccinate synthetase (C) IMP dehydrogenase (D) HGPR Tase

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Description : Insulin has no effect on the activity of the enzyme: (A) Glycogen synthetase (B) Fructokinase (C) Pyruvate kinase (D) Pyruvate dehydrogenase

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