Urea Cycle

Question 1

Ornithine Transcarbamolyase (OT) def

Answer 1

OROTIC ACID buildup. hyperammonemia. Carbamoyl Phosphate+Ornithine=Citrulline. decrease Citrulline.

Question 2

Arginosuccinate synthase def

Answer 2

citrullinemia. citrulline to arginosuccinate. Hyperammonemia. neuro problems common to Urea Cycle Disorders (UCDs)

Question 3

Arginase def

Answer 3

arginemia. arginine to ornithine. hyperammonemia. mental retardation

Question 4

Acetylglutamate synthase def

Answer 4

hyperammonemia, mental retardation, seizures, coma, death

Question 5

Hepatitis A

Answer 5

elevated AST, ALT (liver damage). hyperammonemia. neuro problems common to UCDs. symptoms: disorientation, jerking motions, facial grimacing.

LACTULOSE: reduce ammonia by producing lactic acid, acidifying colon, ionizing ammonia which is not absorbed as well.
low protein diet
also aromatic acids: Benzoate, Phenylbutyrate