Block 3 - AA Degradation and Special Products

Question 1

an amino acid whose carbons are converted to glucose are known as ________ amino acids

Answer 1

glucogenic

Question 2

an amino acid whose carbons are converted to acetyl CoA or acetoacetate are known as ________ amino acids

Answer 2

ketogenic acids

Question 3

is tyrosine glucogenic or ketogenic?

Answer 3

both

Question 4

what are the 2 AA’s that are exclusively ketogenic?

Answer 4

lysine and leucine

Question 5

are non-essential AA’s glucogenic or ketogenic?

Answer 5

glucogenic

Question 6

succinyl CoA, oxaloacetate, fumurate, and pyruvate are all major gloconeogenic or ketoneogenic substrates?

Answer 6

gluconeogenic substrates

Question 7

is acetoacetate and acetyl-CoA a major gluconeogenic substrate?

Answer 7

no, both are major ketogenic substrates

Question 8

what are the 3 amino acids that are part of the pyruvate pathway, and can be directly convert to pyruvate?

Answer 8

cysteine, alanine, and serine

Question 9

what 2 AA’s are indirectly a factor in the pyruvate pathway? what direct AA does it help to form?

Answer 9

threonine is converted to glycine, then glycine to serine, which is then converted to pyruvate

Question 10

deficiency in the ______ _____ _____ leads to nonketotic hyperglycinema

Answer 10

glycine cleavage complex (GCC)

Question 11

how many pathways are there for serine to for pyruvate?

Answer 11

2

Question 12

what 2 AA’s can be considred interchangeable in the pyruvate pathway?

Answer 12

glycine and serine

Question 13

choline and ethanolamine are derived from ______

Answer 13

serine

Question 14

PLP

Answer 14

Pyridoxal phosphate, the active form of vitamin B₆,

Question 15

of the pathways for serine to form pyruvate, the major factor involved in them are _______ _______ in the first, single step, and ______ and ________ used in the other

Answer 15

serine dehydratase, and PLP and 3-phosphoglycerate (3-PG)

Question 16

amino acids that are converted to acetyl Co-A are considered to be _______

Answer 16

ketogenic

Question 17

tyrosine, a conditionally essential AA, is produced from _____

Answer 17

phenylalanine

Question 18

is the synthesis of tyrosine reversible?

Answer 18

no

Question 19

is tyrosine glucogenic or ketogenic?

Answer 19

both

Question 20

oxidation of tyrosine forms what two products?

Answer 20

acetoacetate (ketogenic)and fumurate (glucogenic)

Question 21

Deficiencies in different enzymes of the pathway from phenylalanine to form tyrosine to be oxidized to acetoacetate and fumurate can result in what 3 diseases?

Answer 21

phenylketonuria (PKU), tyrosinemia, and alcaptonuria

Question 22

a defect or deficiency in the conversion of phenylalanine to tyrosine results in what conition?

Answer 22

PKU - phenylketonuria

Question 23

PKU causes accumulation of ________

Answer 23

phenylpyruvate

Question 24

is tryptophan glucogenic or ketogenic?

Answer 24

both

Question 25

the oxidation of tryptophan forms what 4 major things?

Answer 25

alanine, acetyl CoA, formate, and NAD(P)

Question 26

Deficiency in α-ketoacid decarboxylase leads to ______________

Answer 26

Maple syrup urine disease (MSUD)

Question 27

if an AA yields acetyl CoA is it keto- or glucogenic?

Answer 27

ketogenic

Question 28

Valine yields _______

Answer 28

propionyl-CoA

Question 29

Isoleucine yields ________ and __________

Answer 29

acetyl-CoA and propionyl-CoA

Question 30

Leucine yields ________ and __________

Answer 30

acetyl-CoA and acetoacetate

Question 31

deficiencies in what three AA's can result in maple syrup urine disease?

Answer 31

valine, isoleucine, and leucine

Question 32

AAs degraded to form succinyl CoA can/cannot be synthesized in humans

Answer 32

cannot

Question 33

what AA's are degraded to form succinyl CoA?

Answer 33

methionine, threonine, valine, and isoleucine (Val and Iso b/c proprionyl CoA is converted to Succinyl CoA)

Question 34

Propionyl CoA is carboxylated in a biotin-requiring reaction, forming ___________, which is then rearranged in a vitamin B12-requiring reaction, producing succinyl CoA

Answer 34

methylmalonyl CoA

Question 35

Methylmalonyl CoA is rearranged to succinyl CoA in a reaction requiring what vitamin?

Answer 35

vitamin B12

Question 36

in the catabolism of branched AA's the first step is when the AA is converted to ________ by a __________reaction

Answer 36

alpha-ketoacid by transamination

Question 37

in the catabolism of branched AA's the second step is catalyzed by __________

Answer 37

alpha-ketoacid dehydrogenase (or decarboxylase)

Question 38

Histidine, Arginine, Glutamate, Glutamine, and Proline can all be broken down into what?

Answer 38

glutamate 5-semialdehyde

Question 39

Carbons of fumarate are derived from _______

Answer 39

aspartate

Question 40

The major route for the disposal of Asp involves conversion to ________ via transamination, but can form _______ in the urea cycle

Answer 40

OAA, fumurate

Question 41

The first integrase inhibitor-based single tablet regimen use to treat HIV-1 is _________

Answer 41

Stribild