march 14

Question 1

other sources of ammonia

Answer 1

amines from diet and monoamines that serve as hormones/neurotransmitters
- catabolism of purines and pyrimidines

Question 2

ammonia levels kept low by

Answer 2

formation of urea
formation of glutamate in muscle by glutamate dehydrogenase
formation of glutamine in most tissue by glutamine synthetase

Question 3

ammonia toxic to

Answer 3

CNS

Question 4

problems with converting glutamate to glutamine

Answer 4

• glutamine as osmolyte in astrocytes(glial cells) causes uptake of water(brain swelling)
• glutamate is a neurotransmitter and a substrate for synthesis of gaba

Question 5

hyperanemia causes

Answer 5

reflects cns as a target 
- tremors
- slurring of speech 
- blurred vision
- cerebral edema 
- coma
ammonia levels can rise above 1000 mM

Question 6

acquired hyperanemia

Answer 6

viral hepatitis, hepatotoxins

liver is cirrhotic and collateral circulation develops which bypasses the liver

Question 7

congenital hyperanemia

Answer 7

defects in genes that code for all 5 of urea cycle enzyme most deadly
defects in arginase less deadly(contains 3 amino groups can be excreted in urine)
(should inhibit protein intake and increase calorie intake)
(create compounds that bind amino acids and form compounds excreted in urine)

Question 8

glucogenic

Answer 8

aa catabolized to pyruvate of an intermediate of the tca cycle

Question 9

An amino acid is ketogenic if

Answer 9

amino acids catabolized to acetyl coa or acetoacetate

- lead to formation of fatty acids

Question 10

exclusively ketogenic amino acids

Answer 10

lysine and leucine

Question 11

enzyme for valine, leucine and isoleucine

Answer 11

branched chain amino transferase

Question 12

phenylketoria

Answer 12

deficiency in phenylalanine hydroxylase activity

• so increased phenylalanine in tissues, plasma and urine increased(phenyllactate and phenylacetate), phenylpyruvate.
• must have no aspartame