BIOCHEM

Question 1

Impaired formation of N-acetylglutamate. What REACTION is impaired?

Answer 1

CO2+NH3 to carbamoyl phosphate

Question 2

N-acetylglutamate is an essential activator of

Answer 2

CPS1

Question 3

N-acetylglutamate is formed by

Answer 3

the enzyme N-acetylglutamate synthetase from precursors actyl-CoA and glutamate

Question 4

Immediate source of nitrogen for urea in urea cycle

Answer 4

aspartate

Question 5

paraneoplastic syndrome to cerebellar purkinje cell degen. eti of condition?

Answer 5

autoimmune

Question 6

oxaloacetate forms aspartate while reacting with glutamate. What participates?

Answer 6

pyridoxine

Question 7

propionyl CoA is derived from

Answer 7

amino acids Val, Ile, Met, Thr; odd numbered fatty acids, cholesterol side chains

Question 8

propionic acidemia

Answer 8

congenital def of propionyl CoA carboxylase, responsible for prop CoA to methlmalonyl CoA