Amino acid catabolism Flashcards Preview

Enzymes > Amino acid catabolism > Flashcards

Flashcards in Amino acid catabolism Deck (31):
1

Which amino acids are ketogenic?

L and K

2

Which amino acids are broken down into OAA?

N D

3

Which amino acids are broken down into Fumarate?

W Y F

4

Which amino acids are broken down into succinyl-coa?

V I T M

5

Which amino acids can be broken down into aKG?

R H G P

6

Which amino acids are both ketogenic and glucogenic?

T W I F Y

7

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

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

8

What is glutamate dehyrdorgenase regulated by?

It is inhibited by GTP and NADH
It is activated by ADP

9

What is familial hyperinsulinemic hypoglycemia (HHF6)?

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)

10

Which amino acids can be broken down to make pyruvate?

GACS

11

What is Glyoxalate formed from? Go to?

Formed from G, goes to Oxalate

12

What happens to Oxalate? What does it lead to?

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

13

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

THF

14

N gets its ammonium from where?

Ammonium ion

15

Q gets its ammonium from where?

Ammonium ion

16

Biotin is needed for which reaction?

Propionyl-CoA to Succinyl-CoA

17

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

Propionic acidemia

18

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

D-methylmalonic aciduria

19

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

L-Methylmalonic acid uria

20

What protein binds to B12 in the intestine?

Intrinsic factor

21

What transports the intrinsic factor-B12 across the intestines?

Transcolabamin

22

Pernicious anemia is the result of what?

Practical or actual B12 deficiency

23

Megaloblastic anemia is the result of what? Symptoms?

Deficiency of Vit B12 and no Homocystiene to M reaction.
Prevents DNA replication

24

Why does demyelination occur in B12 deficiency?

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

25

Branched chain V is turned to what?

alpha ketoisovalerate, then to propionyl-Coa

26

Branch chain Isoleucine is turned to what?

alpha-keto-beta-methylglutarate

Propionyl-Coa
Acetyl-Coa

27

Branched chain Leucine is converted to what?

alpha-ketoisoproate

Acetyl-Coa
Acetoacetate

28

Tyrosenemia II is a defect in what enzyme? Symptoms?

Tyrosine aminotransferase
Keratitis, photophobia, skin leisions

29

Alkaptonuria is a defect in what enzyme? Symptoms?

Homogentisate oxidase. Black urine, Ochronosis

30

What is the treatment for someone with Alkaptonuria?

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

31

Tyrosenmia I is a defect in what enzyme? Symptoms?

Fumarylacetoacetate hydrolase
Liver and kidney failure due to succinylacetone formation.