Haem synthetase is congenitally deficient in (A) Congenital erythropoietic porphyria (B) Protoporphyria (C) Hereditary coproporphyria (D) Variegate porphyria

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Answer :

Answer : B

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Description : Main symptoms of congenital erythropoietic porphyria is (A) Yellowish teeth (B) Photosensitivity (C) Abdominal pain (D) Brownish urine

Last Answer : Answer : B

Description : The enzyme involved in variegate porphyria is (A) Protoporphyrinogen oxidase (B) Coproporphyrinogen oxidase (C) Uroporphyrinogen decarboxylase (D) ALA decarboxylase

Last Answer : Answer : A

Description : The enzyme involved in congenial erythropoietic porphyria is (A) Uroporphyrinogen I synthase (B) Uroporphyrinogen III cosynthase (C) Protoporphyrinogen oxidase (D) Ferrochelatase

Last Answer : Answer : B

Description : Hereditary coproporphyria is caused due to deficiency of (A) Protoporphyrinogen oxidase (B) ALA synthase (C) ALA dehydratase (D) Coproporphyrinogen oxidase

Last Answer : Answer : D

Description : The regulatory enzyme for haem synthesis is (A) ALA synthetase (B) haem synthetase (C) Both (A) and (B) (D) None of these

Last Answer : Answer : A

Description : In the iron deficient state, transferrin receptors increase in number on the: A. Intestinal mucosal cells B. Erythropoietic cells C. Reticuloendothelial cells D. All of the above

Last Answer : cells B

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

Description : Enzyme deficient in Hyperammonemia type II is (A) Glutamine synthetase (B) Glutaminase (C) Ornithine transcarbamoylase (D) Carbamoylphosphate synthetase

Last Answer : Answer : C

Description : Protoporphyria (erythrohepatic) is characterized by the deficiency of (A) ALA synthase (B) ALA hydratase (C) Protophyrinogen oxidae (D) Ferrochelatase

Last Answer : Answer : D

Description : Iron absorption is hampered by (A) In achlorhydria (B) When ferritin content of intestinal mucosa is low (C) When saturation of plasma transferring is low (D) When erythropoietic activity is increased

Last Answer : Answer : A

Description : During breakdown of haem, the methenyl bridge between the following two pyrrole rings is broken: (A) I and II (B) II and III (C) III and IV (D) IV and I

Last Answer : Answer : A

Description : Regulation of haem synthesis occurs by (A) Covalent modification (B) Repression - derepression (C) Induction (D) Allosteric regulation

Last Answer : Answer : B

Description : The porphyrin present in haem is (A) Uroporphyrin (B) Protoporphyrin I (C) Coproporphyrin (D) Protoporphyrin II

Last Answer : Answer : D

Description : The active transport system for hepatic uptake of bilirubin is congenitally defective in (A) Gilbert’s disease (B) Crigler-Najjar syndrome (C) Rotor’s syndrome (D) Dubin-Johnson syndrome

Last Answer : Answer : A

Description : The characteristic urinary finding in porphyria cutanea tarda is (A) Increased quantity of porphobilinogen (B) Increased quantity of red cell protoporphyrin (C) Increased quantity of uroporphyrin (D) Increased quantity of δ-ALA

Last Answer : Answer : C

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

Last Answer : Answer : A

Description : The characteristic urinary finding in acute intermittent porphyria is (A) Increased quantity of uroporphyrin (B) Increased quantity of coproporphyrin I (C) Increased quantity of coproporphyrin III (D) Massive quantities of porphobilinogen

Last Answer : Answer : D

Description : The major symptom of acute intermittent porphyria includes (A) Abdominal pain (B) Photosensitivity (C) No neuropsychiatric signs (D) Dermatitis

Last Answer : Answer : A

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

Last Answer : Answer : A

Description : Hypervitaminosis K in neonates may cause (A) Porphyria (B) Jaundice (C) Pellagra (D) Prolonged bleeding

Last Answer : Answer : B

Description : Which of the following is a characteristic feature of acute intermittent porphyria? 1) autosomal recessive inheritance 2) excessive faecal protoporphyrin excretion 3) excessive urinary porphobilinigoen between acute attacks 4) hypernatraemia during attacks 5) photosensitivity

Last Answer : Answers-3 Features of acute intermittent porphyria include urinary porphobilinogen excretion raised between attacks, hyponatraemia during an acute attack and autosomal dominant inheritance.

Description : Assertion `:-` Haemoglobin can not get saturated with oxygen more than `97%`. Reason `:-` With a rise in `PO_(2)`, the affinity between `O_(2)` & haem

Last Answer : Assertion `:-` Haemoglobin can not get saturated with oxygen more than `97%`. Reason `:-` With a ... False. D. If both Assertion & Reason are false.

Description : _____ can spread from person to person in respiratory secretions or it may be inherited congenitally. a. Measles b. Chickenpox c. Rosella d. Herpes

Last Answer : c. Rosella

Description : Which of the following is NOT characteristic of Down’s syndrome? A. Decreased neutrophil function B. Macroglossia C. Macrodontia D. An increased susceptibility to periodontal disease E. Congenitally missing teeth

Last Answer : C. Macrodontia

Description : Most congenitally missing teeth are: A. Mandibular 3rd molars B. Mandibular 2nd premolars C. Maxillary lateral incisor

Last Answer : A. Mandibular 3rd molars

Description : What are the commonest congenitally missing teeth:VERY CONTROVERSIAL BUT SEE THE SAMPLE QUESTIONS FROM ADC IT’S THE ANSWER A. 12, 22 B. 35, 45 ACC TO CAMERON C. 15, 25 D. 33, 43

Last Answer : A. 12, 22

Description : Which of the following is NOT characteristic of Down’s syndrome? A. Decreased neutrophil function B. Macroglossia C. Macrodontia D. An increased susceptibility to periodontal disease E. Congenitally missing teeth

Last Answer : C. Macrodontia

Description : Congenital deficiency of ornithine transcarbamoylase causes (A) Hyperammonaemia type I (B) Hyperammonaemia type II (C) Hyperornithinaemia (D) Citrullinaemia

Last Answer : Answer : C

Description : Congenital galactosaemia can lead to (A) Mental retardation (B) Premature cataract (C) Death (D) All of the above

Last Answer : D

Description : Which of the following may be responsible for a hypokalaemic hypertension 1) Non-classical congenital adrenal hyperplasia 2) Barter's syndrome 3) Diabetic nephropathy 4) Liddle's syndrome 5) Type IV renal tubular acidosis

Last Answer : Answers-4 Liddle's syndrome is typically asscoiated with hypokalaemic hypertension and low renin and aldosterone concentrations - the so called pseudo-hyperaldosteronism. Barter's syndrome is associated ... , which may also be produced with diabetic nephropathy. Hence hyperkalaemia is more typical.

Description : Congenital cataract is seen in which condition?

Last Answer : Galactosemia.

Description : Does anyone else on here have or know about Acute Intermittent Porphyria?

Last Answer : answer:Pee in a covered glass, leave it outside on a sunny day – all day. If it turns purple, you’ve got it. Seriously. Not foolproof, but you know… you could always GO TO YOUR DOCTOR IF YOU SUSPECT A SERIOUS CONDITION. What makes you think you have it?

Description : Porphyria?

Last Answer : DefinitionPorphyrias are a group of rare disorders passed down through families, in which an important part of hemoglobin, called heme, is not made properly. Heme is also found in myoglobin, a ... are many different forms of porphyria. The most common type is porphyria cutanea tarda (PCT).Dru

Description : cAMD is destroyed by (A) Adenylate cyclase (B) Phosphodiesterase (C) Synthetase phosphatase (D) Synthetase kinase

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Description : L-glutamic acid is subjected to oxidative deaminition by (A) L-amino acid dehydrogenase (B) L-glutamate dehydrogenase (C) Glutaminase (D) Glutamine synthetase

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Description : Myeloma cells are lacking in (A) TMP synthetase (B) Formyl transferase (C) HGPRT (D) All of these

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Description : All the following statements about charging of tRNA are correct except (A) It is catalysed by amino acyl tRNA synthetase (B) ATP is converted into ADP and Pi in this reaction (C) The enzyme recognizes the tRNA and the amino acid (D) There is a separate enzyme for each tRNA

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Description : All the following statements about primary gout are true except (A) Its inheritance is X-linked recessive (B) It can be due to increased activity of PRPP synthetase (C) It can be ... activity of hypoxanthine guanine phosphoribosyl transferase (D) De novo synthesis of purines is increased in it

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Description : The following abnormality in PRPP synthetase can cause primary gout: (A) High Vmax (B) Low Km (C) Resistance to allosteric inihbition. (D) All of these

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Description : Cytosolic carbamoyl phosphate synthetase is activated by (A) Glutamine (B) PRPP (C) ATP (D) Aspartate

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Description : Cytosolic carbamoyl phosphate synthetase is inhibited by (A) UTP (B) CTP (C) PRPP (D) TMP

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Description : De novo synthesis of pyrimidine nucleotides is regulated by (A) Carbamoyl phosphate synthetase (B) Aspartate transcarbamoylase (C) Both (A) and (B) (D) None of these

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Description : All the enzymes required for de novo synthesis of pyrimidine nucleotides are cytosolic except (A) Carbamoyl phosphate synthetase (B) Aspartate transcarbamoylase (C) Dihydro-orotase (D) Dihydro-orotate dehydrogenase

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Description : CTP synthetase forms CTP from (A) CDP and inorganic phosphate (B) CDP and ATP (C) UTP and glutamine (D) UTP and glutamate

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Description : An enzyme common to de novo synthesis of pyrimidine nucleotides and urea is (A) Urease (B) Carbamoyl phosphate synthetase (C) Aspartate transcarbamoylase (D) Argininosuccinase

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Description : The first reaction unique to purine nucleotide synthesis is catalysed by (A) PRPP synthetase (B) PRPP glutamyl amido transferase (C) Phosphoribosyl glycinamide synthetase (D) Formyl transferase

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Description : AMP is an allosteric inhibitor of (A) PRPP synthetase (B) Adenylosucciante synthetase (C) Both (A) and (B) (D) None of these

Last Answer : Answer : C

Description : GMP is an allosteric inhibitor of all the following except (A) PRPP synthetase (B) PRPP glutamyl amido synthetase (C) IMP dehydrogenase (D) Adenylosuccinate synthetase

Last Answer : Answer : D

Description : An allosteric inhibitor of adenylosuccinate synthetase is (A) AMP (B) ADP (C) GMP (D) GDP

Last Answer : Answer : A

Description : PRPP synthetase is allosterically inhibited by (A) AMP (B) ADP (C) GMP (D) All of these

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