Galactosemia is due the absence of which enzyme?

Galactosemia is due the absence of which enzyme?
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Answer :

Galactose-1-phosphate uridyl transferase. 
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Amylopectinosis is caused due to absence of (A) Debranching enzyme (B) Branching enzyme (C) Acid maltase (D) Glucose-6-phosphatase

Description : Amylopectinosis is caused due to absence of (A) Debranching enzyme (B) Branching enzyme (C) Acid maltase (D) Glucose-6-phosphatase

Last Answer : Answer : B

Cori disease (Limit dextrinosis) is caused due to absence of (A) Branching enzyme (B) Debranching enzyme (C) Glycogen synthase (D) Phosphorylase

Description : Cori disease (Limit dextrinosis) is caused due to absence of (A) Branching enzyme (B) Debranching enzyme (C) Glycogen synthase (D) Phosphorylase

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The best known cause of galactosemia is the deficiency of (A) Galactose 1-phosphate and uridyl transferase (B) Phosphoglucomutase (C) Galactokinase (D) Lactose synthase

Description : The best known cause of galactosemia is the deficiency of (A) Galactose 1-phosphate and uridyl transferase (B) Phosphoglucomutase (C) Galactokinase (D) Lactose synthase

Last Answer : Answer : A

What is the treatment policy in galactosemia?

Description : What is the treatment policy in galactosemia?

Last Answer : Lactose free diet is given for first five years of life.

What are the features of galactosemia?

Description : What are the features of galactosemia?

Last Answer : Congenital cataract, mental retardation, neonatal hypoglycemia, hepatosplenomegaly, positive Benedictís test.

Absence of the enzyme argininosuccinate synthetase causes (A) Argininosuccinic aciduria (B) Hyperargininemia (C) Tricorrhexis nodosa (D) Citrullinemia

Description : Absence of the enzyme argininosuccinate synthetase causes (A) Argininosuccinic aciduria (B) Hyperargininemia (C) Tricorrhexis nodosa (D) Citrullinemia

Last Answer : Answer : A

Hereditary fructose intolerance involves the absence of the enzyme: (A) Aldalose B (B) Fructokinase (C) Triokinase (D) Phosphotriose isomerase

Description : Hereditary fructose intolerance involves the absence of the enzyme: (A) Aldalose B (B) Fructokinase (C) Triokinase (D) Phosphotriose isomerase

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Mc Ardle’s syndrome is characterized by the absence of (A) Liver phosphorylase (B) Muscle phosphorylase (C) Branching enzyme (D) Debranching enzyme

Description : Mc Ardle’s syndrome is characterized by the absence of (A) Liver phosphorylase (B) Muscle phosphorylase (C) Branching enzyme (D) Debranching enzyme

Last Answer : Answer : B

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 : 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

Last Answer : Answer : A

By consumption of milk and milk products some human beings develop intestinal gas and diarrhoea due to absence of which enzyme (a) Lactase (b) Galactase (c) Protease (d) Rennin

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Last Answer : (a) Lactase

What type Galactosemia is sever or most sever?

Description : What type Galactosemia is sever or most sever?

Last Answer : sever type1 classic is the most sever type,....... sever type1 (classic): galactosemia is the most sever galactosemic disorder

What type Galactosemia is sever or most sever?

Description : What type Galactosemia is sever or most sever?

Last Answer : sever type1 classic is the most sever type,....... sever type1 (classic): galactosemia is the most sever galactosemic disorder

Galactosemia?

Description : Galactosemia?

Last Answer : DefinitionGalactosemia is a condition in which the body is unable to use (metabolize) the simple sugar galactose.Alternative NamesGalactose-1-phosphate uridyl transferase deficiency; Galactokinase deficiency; ... with galactosemia are unable to fully break down the simple sugar galactose. Galactos

Select the incorrect statement from the following. (a) Galactosemia is an inborn error of metabolism. (b) Small population size results in random genetic drift in a population. (c) Baldness is a sex-limited trait. (d) Linkage is an exception to the principle of independent assortment in heredity

Description : Select the incorrect statement from the following. (a) Galactosemia is an inborn error of metabolism. (b) Small population size results in random genetic drift in a population. (c) Baldness is a sex-limited trait. (d) Linkage is an exception to the principle of independent assortment in heredity

Last Answer : (c) Baldness is a sex-limited trait.

Lesch-Nyhan syndrome, the sex linked, recessive absence of HGPRTase, may lead to (A) Compulsive self destructive behaviour with elevated levels of urate in serum (B) Hypouricemia due to liver damage (C) Failure to thrive and megaloblastic anemia (D) Protein intolerance and hepatic encephalopathy

Description : Lesch-Nyhan syndrome, the sex linked, recessive absence of HGPRTase, may lead to (A) Compulsive self destructive behaviour with elevated levels of urate in serum (B) Hypouricemia due to liver damage (C) Failure to thrive and megaloblastic anemia (D) Protein intolerance and hepatic encephalopathy

Last Answer : Answer : A

In adrenogenital syndrome due to total absence of 21-hydroxylase in adrenal cortex, there is (A) Deficient secretion of glucocorticoids (B) Deficient secretion of mineralcorticoids (C) Excessive secretion of androgens (D) All of these

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Last Answer : Answer : D

Bassen-Kornzweig syndrome is due to (A) Absence of Apo-C-II (B) Defect in Apo-B synthesis (C) Absence of Apo-E (D) Absence of Apo-D

Description : Bassen-Kornzweig syndrome is due to (A) Absence of Apo-C-II (B) Defect in Apo-B synthesis (C) Absence of Apo-E (D) Absence of Apo-D

Last Answer : Answer : B

Zellweger’s syndrome is due to inherited absence of (A) Peroxisomes (B) Phospholipase A1 (C) Acyl-Co-A dehydrogenase (D) Thiolase

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What will happen if the secretion of parietal cells of gastric glands is blocked with an inhibitor? (a) In the absence of HCl secretion, inactive pepsinogen is not converted into the active enzyme pepsin. (b) Enterokinase will not be released from the duodenal mucosa and so trypsinogen is not converted to trypsin. (c) Gastric juice will be deficient in chymosin. (d) Gastric juice will be deficient in pepsinogen.

Description : What will happen if the secretion of parietal cells of gastric glands is blocked with an inhibitor? (a) In the absence of HCl secretion, inactive pepsinogen is not converted into the active ... c) Gastric juice will be deficient in chymosin. (d) Gastric juice will be deficient in pepsinogen.

Last Answer : (a) In the absence of HCl secretion, inactive pepsinogen is not converted into the active enzyme pepsin.

Which of the following describes the given graph correctly? Potential energy Reaction Product Substrate B A (a) Endothermic reaction with energy A in presence of enzyme and B in absence of enzyme. (b) Exothermic reaction with energy A in presence of enzyme and B in absence of enzyme. (c) Endothermic reaction with energy A in absence of enzyme and B in presence of enzyme. (d) Exothermic reaction with energy A in absence of enzyme and B in presence of enzyme.

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Last Answer : (b) Exothermic reaction with energy A in presence of enzyme and B in absence of enzyme

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 : 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

Last Answer : Answer : A

In humans purine are catabolised to uric acid due to lack of the enzyme: (A) Urease (B) Uricase (C) Xanthine oxidase (D) Guanase

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Neonatal tyrosinemia is due to deficiency of the enzyme: (A) p-Hydroxyphenylpyruvate hydroxylase (B) Fumarylacetoacetate hydrolase (C) Phenylalanine hydroxylase (D) Tyrosine dehydrogenase

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Albinism is due to deficiency of the enzyme: (A) Phenylalanine hydroxylase (B) Tyrosinase (C) p-Hydroxyphenylpyruvic acid oxidase (D) Tyrosine dehydrogenase

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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

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Last Answer : Answer : B

Jamaican vomiting sickness is due to inactivation of the enzyme (A) Pyruvate carboxylase (B) Acyl-Co-A synthetase (C) Acyl-Co-A dehydrogense (D) Thiolase

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

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Last Answer : Answer : D

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

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

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

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Last Answer : Answer : B

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 : 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

Last Answer : Answer : B

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

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

Krabbe’s disease is due to the deficiency of the enzyme: (A) Ceramide lactosidase (B) Ceramidase (C) β-Galactosidase (D) GM1 β-Galactosidase

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Last Answer : Answer : C

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 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

All the following statements about obstructive jaundice are true except (A) Prothrombin time may be prolonged due to impaired absorption of vitamin K (B) Serum alkaline phosphatase may be raised due to increased release of the enzyme from liver cells (C) Bile salts may enter systemic circulation due to biliary obstruction (D) There is no defect in conjugation of bilirubin

Description : All the following statements about obstructive jaundice are true except (A) Prothrombin time may be prolonged due to impaired absorption of vitamin K (B) Serum alkaline phosphatase may be raised ... systemic circulation due to biliary obstruction (D) There is no defect in conjugation of bilirubin

Last Answer : Answer : B

Alkaptonuria occurs due to deficiency of the enzyme: (A) Maleylacetoacetate isomerase (B) Homogentisate oxidase (C) p-Hydroxyphenylpyruvate hydroxylase (D) Fumarylacetoacetate hydrolase

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Last Answer : Answer : B

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 : 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

Last Answer : Answer : D

Tyrosinosis is due to defect in the enzyme: (A) Fumarylacetoacetate hydrolase (B) p-Hydroxyphenylpyruvate hydroxylase (C) Tyrosine transaminase (D) Tyrosine hydroxylase

Description : Tyrosinosis is due to defect in the enzyme: (A) Fumarylacetoacetate hydrolase (B) p-Hydroxyphenylpyruvate hydroxylase (C) Tyrosine transaminase (D) Tyrosine hydroxylase

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

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

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 activity (B) Glucose-6-P Dehydroglucose deficiency leads to disfuction of many tissues (C) G-6-p Dehydroglucose deficiency is due to a single deletion of a large sequence of DNA in the G-6-PD gene (D) G-6-PD deficiency is precipitated by ingestion of drugs such as aspirin

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

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

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 : 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

Last Answer : Answer : D

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

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

Last Answer : Answer : B

Complete absence of hypoxanthine guanine phospharibosyl transferase causes (A) Primary gout (B) Immunodeficiency (C) Uric acid stones (D) Lesh-Nyhan syndrome

Description : Complete absence of hypoxanthine guanine phospharibosyl transferase causes (A) Primary gout (B) Immunodeficiency (C) Uric acid stones (D) Lesh-Nyhan syndrome

Last Answer : Answer : D

Hydrogen peroxide may be detoxified in the absence of an oxygen acceptor by (A) Peroxidase (B) Catalase (C) Both (A) and (B) (D) None of these

Description : Hydrogen peroxide may be detoxified in the absence of an oxygen acceptor by (A) Peroxidase (B) Catalase (C) Both (A) and (B) (D) None of these

Last Answer : Answer : B

The desaturation and chain elongation system of polyunsaturated fatty acids are greatly diminished in the absence of (A) Insulin (B) Glycagon (C) Epinephrine (D) Thyroxine

Description : The desaturation and chain elongation system of polyunsaturated fatty acids are greatly diminished in the absence of (A) Insulin (B) Glycagon (C) Epinephrine (D) Thyroxine

Last Answer : Answer : A

In haemolytic jaundice, urine shows (A) Absence of bile pigments and urobilinogen (B) Presence of bile pigments and urobilinogen (C) Absence of bile pigments and presence of urobilinogen (D) Presence of bile pigments and absence of urobilinogen FATS AND FATTY ACID METABOLISM 91

Description : In haemolytic jaundice, urine shows (A) Absence of bile pigments and urobilinogen (B) Presence of bile pigments and urobilinogen (C) Absence of bile pigments and presence of urobilinogen (D) Presence of bile pigments and absence of urobilinogen FATS AND FATTY ACID METABOLISM 91

Last Answer : Answer : C