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

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Answer :

Answer :  D

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Description : Maple syrup urine diseases is an inborn error of metabolism of (A) Sulphur-containing amino acids (B) Aromatic amino acids (C) Branched chain amino acids (D) Dicarboxylic amino acids

Last Answer : Answer : C

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

Last Answer : Answer : B

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

Description : 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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Description : Selenium is a constituent of the enzyme: (A) Glutathione peroxidase (B) Homogentisate oxidase (C) Tyrosine hydroxylase (D) Phenylalanin hydroxylase

Last Answer : Answer : A

Description : Richner-Hanhart syndrome is due to defect in (A) Tyrosinase (B) Phenylalanine hydroxylase (C) Hepatic tyrosine transaminase (D) Fumarylacetoacetate hydrolase

Last Answer : Answer : B

Description : 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

Last Answer : Answers-5 The following inborn errors of amino acid metabolism are associated with abnormal odours: Glutaric acidaemia type II (sweaty feet), hawkinsinuria (swimming pool), isovaleric acidaemia (sweaty feet), ... The general rule is that if a child smells peculiar he requires a metabolic work-up.

Description : Most of the ammonia released from L-αamino acids reflects the coupled action of transaminase and (A) L-glutamate dehydrogenase (B) L-amino acid oxidase (C) Histidase (D) Serine dehydratase

Last Answer : Answer : A

Description : All the following are true about phenylketonuria except (A) Deficiency of phenylalanine hydroxylase (B) Mental retardation (C) Increased urinary excretion of p-hydroxyphenyl pyruvic acid (D) Decrease serotonin formation

Last Answer : Answer : C

Description : Absence of phenylalanine hydroxylase causes (A) Neonatal tyrosinemia (B) Phenylketonuria (C) Primary hyperoxaluria (D) Albinism

Last Answer : Answer : D

Description : Enzymic deficiency in β-aminoisobutyric aciduria is (A) Adenosine deaminase (B) Xanthine oxidase (C) Orotidylate decarboxylase (D) Transaminase

Last Answer : Answer : D

Description : An autosomal recessive disorder, xanthinuria is due to deficiency of the enzymes: (A) Adenosine deaminase (B) Xanthine oxidase (C) HGPRTase (D) Transaminase

Last Answer : Answer : B

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

Last Answer : Answer : B

Description : Increased urinary indole acetic acid is diagnostic of (A) Maple syrup urine disease (B) Hartnup disease (C) Homocystinuia (D) Phenylketonuria

Last Answer : Answer : B

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

Last Answer : Answer : B

Description : The deficiency of copper decreases the activity of the enzyme: (A) Lysine oxidase (B) Lysine hydroxylase (C) Tyrosine oxidase (D) Proline hydroxylase

Last Answer : Answer : A

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

Last Answer : Answer : A

Description : An important feature of maple syrup urine disease is (A) Patient can not be treated by dietary regulation (B) Without treatment death, of patient may occur by the end of second year of life (C) Blood levels of leucine, isoleucine and serine are increased (D) Excessive brain damage

Last Answer : Answer : D

Description : Maple syrup urine disease becomes evident in extra uterine life by the end of (A) First week (B) Second week (C) Third week (D) Fourth week

Last Answer : Answer : A

Description : In which of the following is mental retardation an expected finding? 1) Alkaptonuria 2) Cystinuria 3) Glycogen storage disease 4) Lactose intolerance 5) Maple syrup urine disease

Last Answer : Answers-5 MENTAL RETARDATION. Fragile X syndrome-commonest male cause. Hypoxia at birth, intaventricular haemorrhage, rhesus disease, Congenital infections - toxoplasmosis, CMV, rubella ... with diet. -homocystinuria, phenylketonuria -maple syrup urine disease, tryptophanuria -galactosaemia

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

Description : Tyrosine hydroxylase is inhibited by (A) Catecholamines (B) α−Methyldopa (C) Phenylalanine (D) Vanillyl mandelic acid

Last Answer : Answer : A

Description : In the B chain of insulin molecule, the Nterminal amino acid is (A) Proline (B) Threonine (C) Phenylalanine (D) Lysine

Last Answer : Answer : C

Description : In A chain of the insulin molecule the Nterminal amino acid is (A) Glycine (B) Valine (C) Serine (D) Phenylalanine

Last Answer : Answer : A

Description : Branched chain amino acids are (A) Cysteine and cystine (B) Tyrosine and Tryptophan (C) Glycine and Serine (D) Valine, Leucine and Isoleucine

Last Answer : Answer : D

Description : All the following are branched chain amino acids except (A) Isoleucine (B) Alanine (C) Leucine (D) Valine

Last Answer : Answer : B

Description : What are branched chain amino acids?

Last Answer : Valine, leucine and isoleucine.

Description : Maple syrup urine disease?

Last Answer : DefinitionMaple syrup urine disease (MSUD) is a metabolism disorder passed down through families in which the body cannot break down certain parts of proteins. Urine in persons with this condition ... . Even in the mildest form, repeated periods of physical stress can cause mental retardationand h

Description : -child , urine odor like burned sugar: - Phenylketonuria - Maple syrup urine disease

Last Answer : Maple syrup urine disease _______________________________

Description : A modified amino acid solution with increased equimolar branched-chain amino acids and decreased aromatic amino acids has been proposed for patients with hepatic insufficiency. Which of the following ... D. In some studies of surgical patients, improvements in mortality have been reported.

Last Answer : Answer: D DISCUSSION: The use of modified amino acid solutions is based on the false neurotransmitter hypothesis of the cause of hepatic coma. According to this hypothesis, the imbalance ... in a group of patients with cirrhosis, decreasing morbidity and showing a trend toward decreased mortality

Description : The rate limiting step in catecholamine synthesis is catalysed by (A) Phenylalanine hydroxylase (B) Tyrosine hydroxylase (C) Dopa decarboxylase (D) Phenylethanolamine N-methyl transferase

Last Answer : Answer : B

Description : For Catecholamine biosynthesis the rate limiting enzyme is (A) DOPA decarboxylase (B) DOPAMINE β-hydroxylase (C) Tyrosine hydroxylase (D) Phenylalanine hydroxylase

Last Answer : Answer : C

Description : Hydroxylation of phenylalanine requires all of the following except (A) Phenylalanine hydroxylase (B) Tetrahydrobiopterin (C) NADH (D) Molecular oxygen

Last Answer : Answer : C

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

Last Answer : Answer : A

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 : The biosynthesis of both Catecholamine and serotonin require (A) Tyrosine hydroxylase (B) N-methyl transferase (C) Aromatic amino acid decarboxylase (D) Tryptophan pyrrolase

Last Answer : Answer : B

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 : Thyroid hormones are synthesized by the iodination of the amino acid: (A) Glycine (B) Phenylalanine (C) Alanine (D) Tyrosine

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Description : Activation or inactivation of certain key regulatory enzymes is accomplished by covalent modification of the amino acid: (A) Tyrosine (B) Phenylalanine (C) Lysine (D) Serine

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Description : The amino acid which synthesizes many hormornes: (A) Valine (B) Phenylalanine (C) Alanine (D) Histidine

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Description : Which one of the following is an essential amino acid? (A) Arginine (B) Tyrosine (C) Phenylalanine (D) Proline

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Description : The amino acid containing an indole ring: (A) Tryptophan (B) Arginine (C) Threonine (D) Phenylalanine

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Description : Seratonin is derived in the body from the following amino acid: (A) Phenylalanine (B) Histidine (C) Tryptophan (D) Serine

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Description : The limiting amino acid of rice is (A) Lysine (B) Tryptophan (C) Phenylalanine (D) Tyrosine

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Description : Million-Nasse’s reaction is specific for the amino acid: (A) Tryptophan (B) Tyrosine (C) Phenylalanine (D) Arginine

Last Answer : Answer : B

Description : 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

Last Answer : Answer : D

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

Last Answer : (A) Carboxylase

Description : Deficiency of biotin results in decrease in (A) Amino acid synthesis (B) Lipid synthesis (C) Kidney (D) Fatty acid synthesis

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Description : I-cell disease results from absence of the following from lysosomal enzymes: (A) Signal sequence (B) Mannose-6-phosphate (C) Sialic acid (D) A serine residue

Last Answer : Answer : D