Metabolic pathway of amino acids

1. A young adult with albinism is found to be at increased risk for skin cancer. Which of the following is the precursor of melanin?

a) Tryptophan

b) Tyrosine

c) Arginine

2. The amino acids involved in the synthesis of creatine are

a) Arginine, glycine, active methionine

b) Arginine, alanine, glycine

c) Glycine, lysine, methionine

d) Arginine, lysine, methionine

3. Source of nitrogen in urea cycle is

a) Glutamate and aspartate

b) GIutamate and NH3

c) Arginine and aspartate

d) NH3and aspartame

4. Ochronosis is seen in:

a) Alkaptonuria

b) Cystinosis

c) Maplesyrup disease

d) Homiocystinuria

5. Which one of the following can be a homologous substitution for isoleucine in a protein sequence?

a) Methionine

b) B Aspartic acid

c) Valine

d) Arginine

6. A compound serving a link between citric acid cycle and urea cycle is

a) Malate

b) Citrate

c) Succinate

d) Fumarate

7. Histidine is degraded to α-ketoglutarate and is described as a

a) Gluco amino acid

b) Glucogenic amino acid

c) Ketogenic amino acid

d) Keto-gluco amino acid

8. Transamination is the process where

a) Carboxyl group is transferred from amino acid

b) α-amino group is removed from the amino acid

c) Polymerisation of amino acid takes place

d) None of the above

9. A person with phenylketonuria is advised not to consume which of the following products?

a) Glycine containing foods

b) Fat containing food

c) Glucose

d) Aspartame

10. In which of the following conditions the level of creatinine kinase 1 increases?

a) Myocardial ischemia

b) Brain Ischemia

c) Kidney damage

d) Electrical cardioversion

11. Source of nitrogen in urea cycle are?

a) Asparatate and ammonia

b) Glutamate and ammonia

c) Arginine and ammonia

d) Uric acid

12. Amino acid carrier defect is found in?

a) Maple syrup urine disease

b) Alkaptonuria

c) Phenylketonurea

d) Cystinuria

13. The amino acid producing ammonia in kidney is

a) Glutamine

b) Alanine

c) Methionine

d) Glycine

14. Ochronosis is an important finding of

a) Tyrosinemia

b) Tyrosinosis

c) Alkaptonuria

d) RichnerHanhart syndrome

15. NH3 is removed from brain mainly by

a) Creatinine formation

b) Uric acid production

c) Urea formation

d) Glutamine formation

16. One of the following amino acid category comes in all glucogenic, both glucogenic and ketogenic?

a) Sulfur containing amino acid

b) OH group containing amino acid

c) Branched chain amino acid

d) Aromatic amino acid

17. A patient has an inherited disorder in which the administration of aspartame could be detrimental to her health. This patient most likely has which of the following genetic disorders?

a) Hyperornithinemia

b) Hyperuricemia

c) Hypervalinemia

d) Phenylketonuria

18. In a 55- year-old man, who has been diagnosed with cirrhosis of liver, Ammonia is not getting detoxified and can damage brain. Which of the following amino acids can covalently bind ammonia, transport and store in a non toxic form?

a) Aspartate

b) Glutamate

c) Serine

d) Cysteine

19. A patient diagnosed with Hart Nup disease, (due to deficiency of transporter required for the absorption of amino acid tryptophan), has been brought with skin rashes and suicidal tendencies. Tryptophan is a precursor for many compounds, the deficiencies of which can cause the said symptoms. Which out of the following compounds is not synthesized by tryptophan?

a) Serotonin

b) Epinephrine

c) Melatonin

d) Niacin

20. A child was brought to pediatric OPD with complaint of passage of black colored urine. A disorder of Phenylalanine metabolism was diagnosed. A low phenylalanine diet and a supplementation of vitamin C were recommended. Which enzyme defect is expected in this child?

a) Phenyl alanine hydroxylase

b) Tyrosine transaminase

c) Homogentisic acid oxidase

d) Hydrolase

21. All the following are true about phenylketonuria except

a) Deficiency of phenylalanine hydroxylase

b) Mental retardation

c) Increased urinary excretion of phenyl acetyl glutamine

d) Increase melanin formation

22. Clinical symptom in urea cycle disorder is

a) Mental retardation

b) Drowsiness

c) Diarrhoea

d) Oedema


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