Gaucher's Disease is associated with the deficiency of : 1) Hexosaminidase A 2) Sphingomyelinase 3) Arylsulphatase-A 4) B- Glucosidase 5) Iduronidase

Gaucher's Disease is associated with the deficiency of : 1) Hexosaminidase A 2) Sphingomyelinase 3) Arylsulphatase-A 4) B- Glucosidase 5) Iduronidase
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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.
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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 due to the deficiency of the enzyme: (A) α-Fucosidase (B) β-Galactosidase (C) β-Glucosidase (D) Sphingomyelinase

Last Answer : Answer : C

Niemann-Pick disease results from deficiency of (A) Ceramidase (B) Sphingomyelinase (C) Arylsulphatase A (D) Hexosaminidase A

Description : Niemann-Pick disease results from deficiency of (A) Ceramidase (B) Sphingomyelinase (C) Arylsulphatase A (D) Hexosaminidase A

Last Answer : Answer : B

Tay-Sachs disease results from inherited deficiency of (A) Arylsulphatase A (B) Hexosaminidase A (C) Sphingomyelinase (D) Ceramidase

Description : Tay-Sachs disease results from inherited deficiency of (A) Arylsulphatase A (B) Hexosaminidase A (C) Sphingomyelinase (D) Ceramidase

Last Answer : Answer : B

The enzyme deficient in metachromatic leukodystrophy is (A) Arylsulphatase A (B) Hexosaminidase A (C) Ceramidase (D) Sphingomyelinase

Description : The enzyme deficient in metachromatic leukodystrophy is (A) Arylsulphatase A (B) Hexosaminidase A (C) Ceramidase (D) Sphingomyelinase

Last Answer : Answer : A

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 : 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

Gaucher’s disease is due to deficiency of the enzyme: (A) Sphingomyelinase (B) Glucocerebrosidase (C) Galactocerbrosidase (D) β-Galactosidase

Description : Gaucher’s disease is due to deficiency of the enzyme: (A) Sphingomyelinase (B) Glucocerebrosidase (C) Galactocerbrosidase (D) β-Galactosidase

Last Answer : Answer : B

Wolman’s disease is due to deficiency of (A) Cholesteryl ester hydrolase (B) Hexosaminidase A (C) α-Fucosidase (D) Arylsulphatase A

Description : Wolman’s disease is due to deficiency of (A) Cholesteryl ester hydrolase (B) Hexosaminidase A (C) α-Fucosidase (D) Arylsulphatase A

Last Answer : Answer : A

Metachromatic leukodystrophy is due to deficiency of enzyme: (A) α-Fucosidase (B) Arylsulphatase A (C) Ceramidase (D) Hexosaminidase A

Description : Metachromatic leukodystrophy is due to deficiency of enzyme: (A) α-Fucosidase (B) Arylsulphatase A (C) Ceramidase (D) Hexosaminidase A

Last Answer : Answer : B

Defective enzyme in Hunter’s syndrome is (A) α-L-iduronidase (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

Last Answer : Answer : B

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 Sandhoff’s disease is (A) α-Fucosidase (B) Hexosaminidase A and B (C) β-Galactosidase (D) β-Glucosidase

Last Answer : Answer : B

Hexosaminidase A is deficient in (A) Tay-Sachs disease (B) Gaucher’s disease (C) Niemann-Pick disease (D) Fabry’s disease

Description : Hexosaminidase A is deficient in (A) Tay-Sachs disease (B) Gaucher’s disease (C) Niemann-Pick disease (D) Fabry’s disease

Last Answer : Answer : A

Hunter’s syndrome results from absence of (A) Hexosaminidase A (B) Iduronate sulphatase (C) Neuraminidase (D) Arylsulphatase B

Description : Hunter’s syndrome results from absence of (A) Hexosaminidase A (B) Iduronate sulphatase (C) Neuraminidase (D) Arylsulphatase B

Last Answer : Answer : B

Niemann-Pick disease is due to deficiency of the enzyme (A) Ceramidase (B) Glucocerebrosidase (C) Galactocerebrosidase (D) Sphingomyelinase

Description : Niemann-Pick disease is due to deficiency of the enzyme (A) Ceramidase (B) Glucocerebrosidase (C) Galactocerebrosidase (D) Sphingomyelinase

Last Answer : Answer : D

Fabry’s disease is due to the deficiency of the enzyme: (A) Ceramide trihexosidase (B) Galactocerebrosidase (C) Phytanic acid oxidase (D) Sphingomyelinase

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

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 : 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

Last Answer : Answer : A

Farber’s disease is due to the deficiency of the enzyme: (A) α-Galactosidase (B) Ceramidase (C) β-Glucocerebrosidase (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

Inherited deficiency of enzyme cerebrosidase produces (A) Fabry’s disease (B) Niemann pick disease (C) Gaucher’s disease (D) Tay-sach’s disease

Description : Inherited deficiency of enzyme cerebrosidase produces (A) Fabry’s disease (B) Niemann pick disease (C) Gaucher’s disease (D) Tay-sach’s disease

Last Answer : Answer : C

Inherited deficiency of β−glucosidase causes (A) Tay-Sachs disease (B) Metachromatic leukodystrophy (C) Gaucher’s disease (D) Multiple sclerosis

Description : Inherited deficiency of β−glucosidase causes (A) Tay-Sachs disease (B) Metachromatic leukodystrophy (C) Gaucher’s disease (D) Multiple sclerosis

Last Answer : Answer : C

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 : 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

Last Answer : Answer : A

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 Krabbe’s disease is (A) Hexosaminidase A(B) Arylsuphatase A (C) β-Galactosidase (D) α-Fucosidase

Last Answer : Answer : C

Serum ferroxidase level decreases in (A) Gaucher’s disease (B) Cirrhosis of liver (C) Acute pancreatitis (D) Wilson’s disease ENZYMES 143

Description : Serum ferroxidase level decreases in (A) Gaucher’s disease (B) Cirrhosis of liver (C) Acute pancreatitis (D) Wilson’s disease ENZYMES 143

Last Answer : Answer : D

Serum lipase level increases in (A) Paget’s disease (B) Gaucher’s disease (C) Acute pancreatitis (D) Diabetes mellitus

Description : Serum lipase level increases in (A) Paget’s disease (B) Gaucher’s disease (C) Acute pancreatitis (D) Diabetes mellitus

Last Answer : Answer : C

The concentration of sphingomyelins are increased in (A) Gaucher’s disease (B) Fabry’s disease (C) Fabrile disease (D) Niemann-Pick disease

Description : The concentration of sphingomyelins are increased in (A) Gaucher’s disease (B) Fabry’s disease (C) Fabrile disease (D) Niemann-Pick disease

Last Answer : Answer : B

The enzyme deficient in Fabry’s disease is (A) α-Galactosidase (B) β-Galactosidase (C) α-Glucosidase (D) β-Glucosidase

Description : The enzyme deficient in Fabry’s disease is (A) α-Galactosidase (B) β-Galactosidase (C) α-Glucosidase (D) β-Glucosidase

Last Answer : Answer : A

Mental retardation occurs in (A) Tay-Sachs disease (B) Gaucher’s disease (C) Niemann-Pick disease (D) All of these

Description : Mental retardation occurs in (A) Tay-Sachs disease (B) Gaucher’s disease (C) Niemann-Pick disease (D) All of these

Last Answer : Answer : D

β -Galactosidase is deficient in (A) Fabry’s disease (B) Krabbe’s disease (C) Gaucher’s disease (D) Metachromatic leukodystrophy

Description : β -Galactosidase is deficient in (A) Fabry’s disease (B) Krabbe’s disease (C) Gaucher’s disease (D) Metachromatic leukodystrophy

Last Answer : Answer : B

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 Hurler’s syndrome is (A) α-L-diuronidase (B) Iduronate sulphatase (C) Arylsulphatase B (D) C-acetyl transferase

Last Answer : Answer : A

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 : 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

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 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

Last Answer : Answer : B

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 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

Amylo 1, 6 glucosidase is called (A) Branching enzyme (B) debranching enzyme (C) Glucantransferase (D) Phosphorylase

Description : Amylo 1, 6 glucosidase is called (A) Branching enzyme (B) debranching enzyme (C) Glucantransferase (D) Phosphorylase

Last Answer : B

A person’s lysosomes lack the enzyme β-glucosidase ?

Description : There is an inherited disease in which a person’s lysosomes lack the enzyme β-glucosidase What are the clinical and biochemical consequences of this deficiency?

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he following antidiabetic drug inhibits intestinal brush border α-glucosidase enzymes: A. Acarbose B. Pioglitazone C. Metformin D. Guargum

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Which of the following enzyme defects is associated with a characteristic body odour? 1) Phenylalanine aminotransferase 2) Galactose0-phosphate-uridyltransferase 3) Ornithine transcarbamylase deficiency 4) Fumaryl acetoacetase 5) Branched chain ketoacid decarboxylase

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Description : Higher rate of lipogenesis is associated with (A) High proportion of carbohydrate in diet (B) Restricted caloric intake (C) High fat diet (D) Deficiency of insulin

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Description : Zinc deficiency occurs commonly in (A) Acrodermatitis enteropathica (B) Wilson’s disease (C) Xeroderma pigmentosum (D) Menke’s disease

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Description : Refsum’s disease is due to deficiency of the enzyme: (A) Pytantate-α-oxidase (B) Glucocerebrosidase (C) Galactocerebrosidase (D) Ceramide trihexosidase

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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

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Description : What is the disease caused by thiamine deficiency? (A) Nycalopia (B) Scurvy (C) Rickets (D) Beriberi

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