ammonia metabolism

Question 1

disease of negative nitrogen balance

Answer 1

anorexia, Kwarshiorkor

Question 2

most important amino acid

Answer 2

Glutamate –> aplha keto glutarate

Question 3

donates N to urea cycle

Answer 3

Aspartate

Question 4

key in gluconeogenesis

Answer 4

Alanine

Question 5

transports N to liver

Answer 5

Glutamine

Question 6

Body uses these fuels in the FED state

Answer 6

fatty acids and carbohydrates

Question 7

Used in FASTED state for energy

Answer 7

amino acids (gluconeogenesis)

Question 8

important in use of amino acisa as fuel

Answer 8

alanine

Question 9

These enzymes pick up free NH4+

Answer 9

Glutamate DH and Glutamine synthase

Question 10

oxaloacetate of ammonia cycle

Answer 10

ornithine

Question 11

key regulatory enzyme of urea cycle

Answer 11

CPSI

Question 12

defect in arginosuccinate synthetase

Answer 12

add arginase –> allows cycle to move

Question 13

defect in argininosuccinate lyase

Answer 13

add excess arginosuccinate lyase - it will get peed out

Question 14

3 enzymes that fix N to carbon

Answer 14

CPSI, glutamate dehydrogenase, glutamine synthase

Question 15

buildup of this amino acid activates this enzyme

Answer 15

arginine - creates NAG

Question 16

all disorders of urea cycle result in this

Answer 16

elevated blood NH4+

Question 17

Disorder with elevated urinary orotic acid due to buildup of carbamoyl phospate

Answer 17

Ornithine Transcarbamoylase enzme def.

Question 18

HHH syndrome, elevated ammonaemia, ornithaemia, citrullinameia

Answer 18

defective transporter

Question 19

substrates of homocitrulline

Answer 19

carbamoyl phosphate and lysine

Question 20

test for ammonia

Answer 20

free ammonia and BUN

Question 21

elevated orotic acid sign of

Answer 21

defect past CPT1, excess carbamoyl phosphate and aspartate

Question 22

make amino acids excreted in urine

Answer 22

benzoic acid (glycine) and Pheylbutyrate (glutamine)