Amino acid catabolism

Question 1

Which amino acids are ketogenic?

Answer 1

L and K

Question 2

Which amino acids are broken down into OAA?

Answer 2

N D

Question 3

Which amino acids are broken down into Fumarate?

Answer 3

W Y F

Question 4

Which amino acids are broken down into succinyl-coa?

Answer 4

V I T M

Question 5

Which amino acids can be broken down into aKG?

Answer 5

R H G P

Question 6

Which amino acids are both ketogenic and glucogenic?

Answer 6

T W I F Y

Question 7

What reaction does glutamate dehydrogenase catalyze? Why is this important?

Answer 7

the reaction from E to aKG. This is important since aKG is needed as an ammonium ion acceptor for aminotransferases.

Question 8

What is glutamate dehyrdorgenase regulated by?

Answer 8

It is inhibited by GTP and NADH

It is activated by ADP

Question 9

What is familial hyperinsulinemic hypoglycemia (HHF6)?

Answer 9

Hypoglycemiaand hyperammonemia after high protein meals due to caused by an insensitivity of Glutamate dehydro to GTP. Also reduces N-acetylglutamate synthesis (activator of urea cycle)

Question 10

Which amino acids can be broken down to make pyruvate?

Answer 10

GACS

Question 11

What is Glyoxalate formed from? Go to?

Answer 11

Formed from G, goes to Oxalate

Question 12

What happens to Oxalate? What does it lead to?

Answer 12

It is a garbage molecule excreted through kidneys. Attaches to Ca, causes renal stones

Question 13

G is cleaved in the mitochondira by what important carbon acceptor?

Answer 13

THF

Question 14

N gets its ammonium from where?

Answer 14

Ammonium ion

Question 15

Q gets its ammonium from where?

Answer 15

Ammonium ion

Question 16

Biotin is needed for which reaction?

Answer 16

Propionyl-CoA to Succinyl-CoA

Question 17

Defects in Propionyl-Coa caboxylase will result in what condition?

Answer 17

Propionic acidemia

Question 18

Defects in racemase in the propionyl-CoA rxn will result in what condition?

Answer 18

D-methylmalonic aciduria

Question 19

Defects in mutase in the propionyl-CoA will result in what condition?

Answer 19

L-Methylmalonic acid uria

Question 20

What protein binds to B12 in the intestine?

Answer 20

Intrinsic factor

Question 21

What transports the intrinsic factor-B12 across the intestines?

Answer 21

Transcolabamin

Question 22

Pernicious anemia is the result of what?

Answer 22

Practical or actual B12 deficiency

Question 23

Megaloblastic anemia is the result of what? Symptoms?

Answer 23

Deficiency of Vit B12 and no Homocystiene to M reaction.

Prevents DNA replication

Question 24

Why does demyelination occur in B12 deficiency?

Answer 24

Impairment of the Methylmalonyl-CoA reaction (used to think, now different)

Question 25

Branched chain V is turned to what?

Answer 25

alpha ketoisovalerate, then to propionyl-Coa

Question 26

Branch chain Isoleucine is turned to what?

Answer 26

alpha-keto-beta-methylglutarate

Propionyl-Coa
Acetyl-Coa

Question 27

Branched chain Leucine is converted to what?

Answer 27

alpha-ketoisoproate

Acetyl-Coa
Acetoacetate

Question 28

Tyrosenemia II is a defect in what enzyme? Symptoms?

Answer 28

Tyrosine aminotransferase

Keratitis, photophobia, skin leisions

Question 29

Alkaptonuria is a defect in what enzyme? Symptoms?

Answer 29

Homogentisate oxidase. Black urine, Ochronosis

Question 30

What is the treatment for someone with Alkaptonuria?

Answer 30

lower F and Y levels, and admin Nitisione (inhibits p-hydoxyphenylpyruvate)

Question 31

Tyrosenmia I is a defect in what enzyme? Symptoms?

Answer 31

Fumarylacetoacetate hydrolase

Liver and kidney failure due to succinylacetone formation.