Amino Acid Metabolism

Question 1

Enzyme that aminates glutamate to form glutamine.

Answer 1

Glutamine synthethase

Question 2

Major carrier of excess nitrogen from tissues

Answer 2

Glutamine

Question 3

Enzyme that deaminates glutamine

Answer 3

Glutaminase

Question 4

Vitamin required by aminotransferases

Answer 4

Pyridoxine

Question 5

Rate-limiting enzyme in the Urea Cycle

Answer 5

Carbamoyl Phosphate Synthetase-1

Question 6

Substrates for Carbamoyl Phosphate

Answer 6

NH4 HCO3 2ATP

Question 7

Added with Carbamoyl Phosphate to produce Citrulline

Answer 7

Aspartate

Question 8

Deficiency of this urea cycle enzyme is distinguished by increase in orotic acid and uracil

Answer 8

ornithine transcarbamoylase

Question 9

Deficient enzyme in Phenylketonuria

Answer 9

phenylalanine hydroxylase

Question 10

Deficient enzyme in Alcaptonuria

Answer 10

homogentisate oxidase

Question 11

N-aspartyl-phenylalanine methyl ester, an artificial sweeter, is also known as

Answer 11

aspartame

Question 12

Accumulation of this substance in the urine makes the urine to gradually darken upon exposure to air

Answer 12

Alcaptonuria

Question 13

The three branched-chain aminoacids

Answer 13

Valine leucine isoleucine

Question 14

Enzyme deficiency in Maple Syrup Urine Disease

Answer 14

Branched chain ketoacid dehydrogenase

Question 15

Genetic disease caused by deficiency in Cystathionine synthase or homocysteine methyl transferase

Answer 15

Homocysteinuria

Question 16

Vitamin deficiency associated with homocysteinemia

Answer 16

Vit B6 deficiency

Question 17

Homocysteinemia and megaloblastic anemia is both deficient in what vitamins

Answer 17

B12, folate

Question 18

Rate-limiting enzyme in Heme Synthesis

Answer 18

Aminolevulinate synthase

Question 19

Common histopathologic finding in B6 deficiency and Lead poisoning

Answer 19

Ringed sideroblasts in bone marrow

Question 20

“Port-wine urine” accompanied by acute onset neurologic and hepatic symptoms

Answer 20

Acute intermittend porphyria

Question 21

Most common porphyria, presenting with photosensitivity and “red-brown urine”

Answer 21

Porphyria cutanea tarda