Description : The enzyme deficient in metachromatic leukodystrophy is (A) Arylsulphatase A (B) Hexosaminidase A (C) Ceramidase (D) Sphingomyelinase
Last Answer : Answer : A
Description : Wolman’s disease is due to deficiency of (A) Cholesteryl ester hydrolase (B) Hexosaminidase A (C) α-Fucosidase (D) Arylsulphatase A
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 : 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
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
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
Last Answer : Answer : D
Description : Inherited deficiency of β−glucosidase causes (A) Tay-Sachs disease (B) Metachromatic leukodystrophy (C) Gaucher’s disease (D) Multiple sclerosis
Description : Gaucher’s disease is due to the deficiency of the enzyme: (A) α-Fucosidase (B) β-Galactosidase (C) β-Glucosidase (D) Sphingomyelinase
Description : Gaucher's Disease is associated with the deficiency of : 1) Hexosaminidase A 2) Sphingomyelinase 3) Arylsulphatase-A 4) B- Glucosidase 5) Iduronidase
Last Answer : Answers-4 Hexosaminidase A deficiency is associated Tay-Sachs disease. Sphingomyelinase deficiency is associated with Niemann-Pick disease. Arylsulphatase-A deficiency is associated with metachromic leucodystrophy.Iduronidase deficiency is associated with Hurlers syndrome.
Description : β -Galactosidase is deficient in (A) Fabry’s disease (B) Krabbe’s disease (C) Gaucher’s disease (D) Metachromatic leukodystrophy
Description : Hunter’s syndrome results from absence of (A) Hexosaminidase A (B) Iduronate sulphatase (C) Neuraminidase (D) Arylsulphatase B
Description : Niemann-Pick disease is due to deficiency of the enzyme (A) Ceramidase (B) Glucocerebrosidase (C) Galactocerebrosidase (D) Sphingomyelinase
Description : Krabbe’s disease is due to the deficiency of the enzyme: (A) Ceramide lactosidase (B) Ceramidase (C) β-Galactosidase (D) GM1 β-Galactosidase
Description : Defective enzyme in Hurler’s syndrome is (A) α-L-diuronidase (B) Iduronate sulphatase (C) Arylsulphatase B (D) C-acetyl transferase
Description : Defective enzyme in Hunter’s syndrome is (A) α-L-iduronidase (B) Iduronate sulphatase (C) Arylsulphatase B (D) C-acetyl transferase
Description : Refsum’s disease is due to deficiency of the enzyme: (A) Pytantate-α-oxidase (B) Glucocerebrosidase (C) Galactocerebrosidase (D) Ceramide trihexosidase
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 : The enzyme ceramidase is deficient in (A) Farber’s disease (B) Fabry’s disease (C) Sandhoff’s disease(D) Refsum’s disease
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
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 : Ceramidase is deficient in (A) Fabry’s disease (B) Farber’s disease (C) Krabbe’s disease (D) Tay-Sachs disease
Description : Hexosaminidase A is deficient in (A) Tay-Sachs disease (B) Gaucher’s disease (C) Niemann-Pick disease (D) Fabry’s disease
Description : Neonatal tyrosinemia is due to deficiency of the enzyme: (A) p-Hydroxyphenylpyruvate hydroxylase (B) Fumarylacetoacetate hydrolase (C) Phenylalanine hydroxylase (D) Tyrosine dehydrogenase
Description : Albinism is due to deficiency of the enzyme: (A) Phenylalanine hydroxylase (B) Tyrosinase (C) p-Hydroxyphenylpyruvic acid oxidase (D) Tyrosine dehydrogenase
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
Description : Acute hemolytic episode after administration of antimalarial, primaquin, is due to deficiency of the enzyme: (A) 6-Phosphogluconate dehydrogenase (B) Glucose-6-phosphate dehydrogenase (C) Epimerase (D) Transketolase
Description : Acute hemolytic anemia in person’s sensitive to the Fava beans is due to the deficiency of the enzyme: (A) Pyruvate dehydrogenase (B) G-6-PD (C) Aconitase (D) Transketolase
Description : Fabry’s disease is due to the deficiency of the enzyme: (A) Ceramide trihexosidase (B) Galactocerebrosidase (C) Phytanic acid oxidase (D) Sphingomyelinase
Description : Alkaptonuria occurs due to deficiency of the enzyme: (A) Maleylacetoacetate isomerase (B) Homogentisate oxidase (C) p-Hydroxyphenylpyruvate hydroxylase (D) Fumarylacetoacetate hydrolase
Description : Gaucher’s disease is due to deficiency of the enzyme: (A) Sphingomyelinase (B) Glucocerebrosidase (C) Galactocerbrosidase (D) β-Galactosidase
Description : Which one of the following statements concerning glucose-6-phosphate dehydrogenase deficiency is correct? (A) Young R.B.Cs, particularly reticulocytes, contain the highest enzyme activity cells show less enzyme ... PD gene (D) G-6-PD deficiency is precipitated by ingestion of drugs such as aspirin
Description : Lack of Glucocorticoids and mineral corticoids might be consequence of which of the following defects in the adrenal cortex? (A) Androstenadione deficiency (B) Estrone deficiency (C) 17 α-OH progesterone deficiency (D) C- α-Hydroxylase deficiency
Description : The defect in adrenal cortex responsible for lack of glucocorticoids and mineralcorticoids is (A) Androstenedione deficiency (B) 17 α -OH progesterone deficiency (C) C-21 hydroxylase deficiency (D) Testosterone deficiency HORMONE METABOLISM 209
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 : Methylene blue staining of metachromatic granules is diagnostic for which of the following bacteria? a. Mycobacterium Tuberculosis b. Coiynebacterium Diphtheriae c. Chlamydia Pneumoniae d. Bordetella Pertussis
Last Answer : b. Coiynebacterium Diphtheriae
Description : An enzyme in saliva which hydrolyzes starch is (A) Pepsinogen (B) Chymotrysin (C) α-Amylase (D) Malate
Description : FAD containing enzyme, catalyzing formation of α, β unsaturated fatty acyl CoA derivative. (A) Acyl CoA dehydrogenase (B) Enoyl hydrase (C) β-OH acyl CoA dehydrogenase (D) Thiolase
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 : The hexose monophosphate pathway includes the enzyme: (A) Maltase dehydrogenase (B) Hexokinase (C) α-Ketoglutarate dehydrogenase (D) Glucose-6-phosphate dehydrogenase
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
Description : In TCA cycle, oxalosuccinate is converted to α-ketoglutarate by the enzyme: (A) Fumarase (B) Isocitrate dehydrogenase (C) Aconitase (D) Succinase
Description : An enzyme of the citric acid cycle also found outside the mitochondria is (A) Isocitrate dehydrogenase (B) Citrate synthetase (C) α-Ketoglutarate dehydrogenase (D) Malate dehydrogenase
Description : In biotin-containing enzymes, the biotin is bound to the enzyme by (A) An amide linkage to carboxyl group of glutamine (B) A covalent bond with CO2 (C) An amide linkage to an amino group of lysine (D) An amide linkage to α-carboxyl group of protein
Description : The enzyme deficient in Fabry’s disease is (A) α-Galactosidase (B) β-Galactosidase (C) α-Glucosidase (D) β-Glucosidase
Description : The α-ketoacid is decarboxylated by H2O2 forming a carboxylic acid with one carbon atom less in the absence of the enzyme: (A) Catalase (B) Decarboxylase (C) Deaminase (D) Phosphatase
Description : All are true about lesch-nyhan syndrome except (A) Produces self-mutilation (B) Genetic deficiency of the enzyme (C) Elevated levels of uric acid in blood (D) Inheritance is autosomal recessive
Description : Orotic aciduria type II reflects the deficiency of the enzyme: (A) Orotate phosphoribosyl transferase (B) Orotidylate decarboxylase (C) Dihydroorotase (D) Dihydroorotate dehydrogenase