Description : Refsum’s disease is due to deficiency of the enzyme: (A) Pytantate-α-oxidase (B) Glucocerebrosidase (C) Galactocerebrosidase (D) Ceramide trihexosidase
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 : 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 : 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
Last Answer : Answer : B
Description : Gaucher’s disease is due to the deficiency of the enzyme: (A) α-Fucosidase (B) β-Galactosidase (C) β-Glucosidase (D) Sphingomyelinase
Last Answer : Answer : C
Description : Gaucher’s disease is due to deficiency of the enzyme: (A) Sphingomyelinase (B) Glucocerebrosidase (C) Galactocerbrosidase (D) β-Galactosidase
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 : Inherited deficiency of enzyme cerebrosidase produces (A) Fabry’s disease (B) Niemann pick disease (C) Gaucher’s disease (D) Tay-sach’s disease
Description : Albinism is due to deficiency of the enzyme: (A) Phenylalanine hydroxylase (B) Tyrosinase (C) p-Hydroxyphenylpyruvic acid oxidase (D) Tyrosine dehydrogenase
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 : 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 : Alkaptonuria occurs due to deficiency of the enzyme: (A) Maleylacetoacetate isomerase (B) Homogentisate oxidase (C) p-Hydroxyphenylpyruvate hydroxylase (D) Fumarylacetoacetate hydrolase
Description : Protein deficiency disease is known as (A) Cushing’s disease (B) Fabry’s disease (C) Parkinson’s disease (D) Kwashiorkor and marasmus
Description : The enzyme ceramidase is deficient in (A) Farber’s disease (B) Fabry’s disease (C) Sandhoff’s disease(D) Refsum’s disease
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 enzyme deficient in Fabry’s disease is (A) α-Galactosidase (B) β-Galactosidase (C) α-Glucosidase (D) β-Glucosidase
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 : 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 : In humans purine are catabolised to uric acid due to lack of the enzyme: (A) Urease (B) Uricase (C) Xanthine oxidase (D) Guanase
Description : The enzyme deficient in metachromatic leukodystrophy is (A) Arylsulphatase A (B) Hexosaminidase A (C) Ceramidase (D) Sphingomyelinase
Description : All the following can be oxidized by βoxidation except (A) Palmitic acid (B) Phytanic acid (C) Linoleic acid (D) Fatty acids having an odd number of carbon atoms
Description : An important feature of Zellweger ’s syndrome is (A) Hypoglycemia (B) Accumulation of phytanic acid in tissues (C) Skin eruptions (D) Accumulation of C26-C38 polyenoic acid in brain tissues
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 : 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 : Schardinger’s enzyme is (A) Lactate dehydrogenase (B) Xanthine dehydrogenase (C) Uric oxidase (D) L amino acid dehydrogenase
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
Description : Ceramidase is deficient in (A) Fabry’s disease (B) Farber’s disease (C) Krabbe’s disease (D) Tay-Sachs disease
Description : The concentration of sphingomyelins are increased in (A) Gaucher’s disease (B) Fabry’s disease (C) Fabrile disease (D) Niemann-Pick disease
Description : Hexosaminidase A is deficient in (A) Tay-Sachs disease (B) Gaucher’s disease (C) Niemann-Pick disease (D) Fabry’s disease
Description : β -Galactosidase is deficient in (A) Fabry’s disease (B) Krabbe’s disease (C) Gaucher’s disease (D) Metachromatic leukodystrophy
Description : An important finding of Fabry’s disease is (A) Skin rash (B) Exophthalmos (C) Hemolytic anemia (D) Mental retardation
Description : Farber’s disease is due to the deficiency of the enzyme: (A) α-Galactosidase (B) Ceramidase (C) β-Glucocerebrosidase (D) Arylsulphatase 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 : Cerebronic acid is present in (A) Glycerophospholipids (B) Sphingophospholipids (C) Galactosyl ceramide (D) Gangliosides
Description : Glycosphingolipids are a combination of (A) Ceramide with one or more sugar residues (B) Glycerol with galactose (C) Sphingosine with galactose (D) Sphingosine with phosphoric acid
Description : Tarui disease is characterized by the deficiency of the enzyme: (A) Liver phosphorylase (B) Muscle phosphorylase (C) Muscle and erythrocyte phosphofructokinase (D) Lysosomal acid maltase
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 : 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