Description : Fabry’s disease is due to the deficiency of the enzyme: (A) Ceramide trihexosidase (B) Galactocerebrosidase (C) Phytanic acid oxidase (D) Sphingomyelinase
Last Answer : Answer : A
Description : Niemann-Pick disease is due to deficiency of the enzyme (A) Ceramidase (B) Glucocerebrosidase (C) Galactocerebrosidase (D) Sphingomyelinase
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.
Last Answer : Answer : B
Description : An important finding in Refsum’s disease is (A) Accumulation of ceramide trihexoside in the kidney (B) Accumulation of phytanic acid in the blood and tissues (C) Accumulation of gangliosides in brain and spleen (D) Skin eruptions
Description : Gaucher’s disease is due to deficiency of the enzyme: (A) Sphingomyelinase (B) Glucocerebrosidase (C) Galactocerbrosidase (D) β-Galactosidase
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 : Krabbe’s disease is due to the deficiency of the enzyme: (A) Ceramide lactosidase (B) Ceramidase (C) β-Galactosidase (D) GM1 β-Galactosidase
Last Answer : Answer : C
Description : Neimann-Pick disease is due to the deficiency of the enzyme: (A) Hexosaminidase A and B (B) Ceramidase (C) Ceramide lactosidase (D) Sphingomyelinase CHAPTER 6 CHAPTER 6 ENZYMES
Description : The enzyme ceramidase is deficient in (A) Farber’s disease (B) Fabry’s disease (C) Sandhoff’s disease(D) Refsum’s disease
Description : Gaucher’s disease is due to the deficiency of the enzyme: (A) α-Fucosidase (B) β-Galactosidase (C) β-Glucosidase (D) Sphingomyelinase
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 : Albinism is due to deficiency of the enzyme: (A) Phenylalanine hydroxylase (B) Tyrosinase (C) p-Hydroxyphenylpyruvic acid oxidase (D) Tyrosine dehydrogenase
Description : Alkaptonuria occurs due to deficiency of the enzyme: (A) Maleylacetoacetate isomerase (B) Homogentisate oxidase (C) p-Hydroxyphenylpyruvate hydroxylase (D) Fumarylacetoacetate hydrolase
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 deficiency of copper decreases the activity of the enzyme: (A) Lysine oxidase (B) Lysine hydroxylase (C) Tyrosine oxidase (D) Proline hydroxylase
Description : Metachromatic leukodystrophy is due to deficiency of enzyme: (A) α-Fucosidase (B) Arylsulphatase A (C) Ceramidase (D) Hexosaminidase A
Description : Refsum’s disease results from a defect in the following pathway except (A) Alpha-oxidation of fatty acids (B) Beta-oxidation of fatty acids (C) Gamma-oxidation of fatty acids (D) Omega-oxidation of fatty acids
Description : Maple syrup urine disease results from absence or serve deficiency of (A) Homogentisate oxidase (B) Phenylalanine hydroxylase (C) Branched chain amino acid transaminase (D) None of these
Description : Wolman’s disease is due to deficiency of (A) Cholesteryl ester hydrolase (B) Hexosaminidase A (C) α-Fucosidase (D) Arylsulphatase A
Description : An autosomal recessive disorder, xanthinuria is due to deficiency of the enzymes: (A) Adenosine deaminase (B) Xanthine oxidase (C) HGPRTase (D) Transaminase
Description : Hereditary coproporphyria is caused due to deficiency of (A) Protoporphyrinogen oxidase (B) ALA synthase (C) ALA dehydratase (D) Coproporphyrinogen oxidase
Description : Acute intermittent porphyria (paraoxymal porphyria) is caused due to deficiency of (A) Uroporphyrinogen I synthase (B) ALA synthase (C) Coproporphyrinogen oxidase (D) Uroporphyrinogen decarboxylase
Description : Ehlers-Danlos syndrome characterized by hypermobile joints and skin abnormalities is due to (A) Abnormality in gene for procollagen (B) Deficiency of lysyl oxidase (C) Deficiency of prolyl hydroxylase (D) Deficiency of lysyl hydroxylase
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 : In humans purine are catabolised to uric acid due to lack of the enzyme: (A) Urease (B) Uricase (C) Xanthine oxidase (D) Guanase
Description : Von Gierke’s disease is characterized by the deficiency of (A) Glucose-6-phosphatase (B) α -1→ 4 Glucosidase (C) 1→ 6 Glucosidase (D) Liver phosphorylase
Description : The enzyme deficient in Sandhoff’s disease is (A) α-Fucosidase (B) Hexosaminidase A and B (C) β-Galactosidase (D) β-Glucosidase
Description : The enzyme deficient in Krabbe’s disease is (A) Hexosaminidase A(B) Arylsuphatase A (C) β-Galactosidase (D) α-Fucosidase
Description : The enzyme deficient in Fabry’s disease is (A) α-Galactosidase (B) β-Galactosidase (C) α-Glucosidase (D) β-Glucosidase
Description : Hypouricaemia can occur in (A) Xanthine oxidase deficiency (B) Psoriasis (C) Leukaemia (D) None of these
Description : Enzymic deficiency in β-aminoisobutyric aciduria is (A) Adenosine deaminase (B) Xanthine oxidase (C) Orotidylate decarboxylase (D) Transaminase
Description : The probable cause of porphyria cutanea tarda is deficiency of (A) Uroporphyrinogen oxidase (B) Coproporphyrinogen oxidase (C) Protoporphyrinogen oxidase (D) Uroporphyrinogen I synthase MINERAL METABOLISM 185
Description : Pompe ’s d isease is caused due to deficiency of (A) Lysosomal α-1→4 and 1→6-glucosidase (B) Glucose-6-phosphatase (C) Glycogen synthase (D) Phosphofructokinase
Description : A drug which prevents uric acid synthesis by inhibiting the enzyme Xanthine oxidase is (A) Aspirin (B) Allopurinal (C) Colchicine (D) Phenyl benzoate
Description : An enzyme involved in catabolism of catecholamines is (A) Dopa decarboxylase (B) Aromatic amino acid decarboxylase (C) Monoamine oxidase (D) Catechol oxidas
Description : Selenium is a constituent of the enzyme: (A) Glutathione peroxidase (B) Homogentisate oxidase (C) Tyrosine hydroxylase (D) Phenylalanin hydroxylase
Description : The enzyme involved in variegate porphyria is (A) Protoporphyrinogen oxidase (B) Coproporphyrinogen oxidase (C) Uroporphyrinogen decarboxylase (D) ALA decarboxylase
Description : The enzyme involved in congenial erythropoietic porphyria is (A) Uroporphyrinogen I synthase (B) Uroporphyrinogen III cosynthase (C) Protoporphyrinogen oxidase (D) Ferrochelatase
Description : Conversion of uroporphyrinogen III to coprophyrinogen III is catalysed by the enzyme.: (A) Uroporphyrinogen decarboxylase (B) Coproporphyrinogen oxidase (C) Protoporphyrinogen oxidase (D) Ferrochelatase
Description : Schardinger’s enzyme is (A) Lactate dehydrogenase (B) Xanthine dehydrogenase (C) Uric oxidase (D) L amino acid dehydrogenase
Description : The pentose phosphate pathway protects erythrocytes against hemolysis by assisting the enzyme: (A) Superoxide dismutase (B) Catalase (C) Glutathionic peroxidase (D) Cytochrome oxidase ENZYMES 149
Description : An enzyme which uses hydrogen acceptor as substrate is (A) Xanthine oxidase (B) Aldehyde oxidase (C) Catalase (D) Tryptophan oxygenase
Description : The enzyme using some other substance, not oxygen as hydrogen acceptor is (A) Tyrosinase (B) Succinate dehydrogenase (C) Uricase (D) Cytochrome oxidase
Description : An enzyme catalyzing oxidoreduction, using oxygen as hydrogen acceptor is (A) Cytochrome oxidase (B) Lactate dehydrogenase (C) Malate dehydrogenase (D) Succinate dehydrogenase
Description : Example of an extracellular enzyme is (A) Lactate dehydrogenase (B) Cytochrome oxidase (C) Pancreatic lipase (D) Hexokinase
Description : Vitamin K is involved in posttranslational modification of the blood clotting factors by acting as cofactor for the enzyme: (A) Carboxylase (B) Decarboxylase (C) Hydroxylase (D) Oxidase
Description : The defective enzyme in histidinemia is (A) Histidine carboxylase (B) Histidine decarboxylase (C) Histidase (D) Histidine oxidase
Description : The enzyme dopamine β-oxidase which catalyses conversion of dopamine to norepinephrine requires (A) Vitamin A (B) Vitamin C (C) Vitamin E (D) Vitamin B12
Description : Which of the following is not an enzyme involved in glycolysis? (A) Euolase (B) Aldolose (C) Hexokinase (D) Glucose oxidase
Last Answer : D
Description : A drug which prevents uric acid synthesis by inhibiting the enzyme xanthine oxidase is (A) Aspirin (B) Allopurinol (C) Colchicine (D) Probenecid
Last Answer : B