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Flashcards in Amino Acid Metabolism Deck (91)
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1

ammonia toxicity is seen in (2)

liver failure

inborn errors of metabolism

2

Effects of ammonia intoxicatio in inborn errors of metabolism n are primarily

 on the nervous system, leading to dizziness, coma, convulsions

3

ammonia toxicity in liver failure is tx with

dietary restriction

eat as little protein as possible, only essential aa

4

Symptoms of Ammonia Toxicity include:

Irritability


V omiting


Lethargy & confusion

 Respiratory distress

Migraines

5

POSSIBLE MECHANISMS FOR AMMONIA TOXICITY 

Brain glutamate dehydrogenase forms glutamate from _____

α-ketoglutarate and NH3

this lower pools of α-ketoglutarate

6

effect of lowered pool of α-ketoglutarate after it is converted with nh3 to glutamate

flux through tca cycle is reduced

7

POSSIBLE MECHANISMS FOR AMMONIA TOXICITY

Increased glutamate leads to increased ______ from the excess NH

inc glutamine

8

POSSIBLE MECHANISMS FOR AMMONIA TOXICITY

. Increased glutamate leads to increased glutamine from the excess NH3 ----> effect 

brain pools of glutamate decrease (affecting NT glutamate levels and gaba)

9

POSSIBLE MECHANISMS FOR AMMONIA TOXICITY

Glutamine leaves brain neurons in exchange for ____

tryptophan.

10

POSSIBLE MECHANISMS FOR AMMONIA TOXICITY

Glutamine leaves brain neurons in exchange for tryptophan ----> 

Trp is converted to serotonin ---> high serotonin thought to be cause of coma in liver failure

11

____ is thought to be an important factor in coma associated with liver failure.

High serotonin

12

possible cause of ammonia toxicity in the brain 

Uncontrolled changes in the levels of 3 neurotransmitters (glutamate, GABA and serotonin) in the brain.

13

The degradation of the AA carbon skeletons involves the removal of ___ and disposal of ___

1. removal of nitrogen

2. disposal of carbon skeletons

14

Ketogenic- amino acids are degraded to either

 acetyl coenzyme A or acetoacetyl CoA

15

 Ketogenic- amino acids are degraded to either acetyl coenzyme A or acetoacetyl CoA, which give rise to ____

ketone bodies 

16

Glucogenic- amino acids are degraded to ___

pyruvate or citric acid cycle intermediates,

17

 Glucogenic- amino acids are degraded to pyruvate or citric acid cycle intermediates, which can give rise to ____ via formation of ____

glucose via formation of phosphoenolpyruvate.

18

_____ amino acids can provide an excellent source of glucose after glycogen stores are gone.

Glucogenic

19

can the  carbons in ketones and acetyl-CoA can or cannot be converted into glucose,

cannot be converted into glucose

can only be converted into FA

20

- Some amino acids can be degraded into multiple intermediates which can be classified as

ketogenic and glucogenic.
[I, F, Y, W, T]

21

amino acids that are exlcusively ketogenic 

leucine
lysine

22

 five amino acids are both ketogenic and glucogenic:
 

isoleucine
phenylalanine
tryptophan
tyrosine
threonine

23

amino acid degradation

Alanine is converted to pyruvate by transamination with α-ketoglutarate by 

alanine transaminase

24

 Serine is converted to pyruvate by ____ by serine dehydratase.

direct deaminatio

25

Cysteine can be converted to pyruvate by a ___

desulfhydrase.

26

Glycine  is converted by____  to serine

 

 serine hydroxymethyltransferase

27

Glycine  is converted by serine hydroxymethyltransferase to _____

 

serine

28

Glycine  is converted by serine hydroxymethyltransferase to serine, which is then converted to ____

 

pyrvuate

29

how to get from glycine to pyrvuate?

glyceine ---> serine ---> pyruvate

30

Glycine  is converted by serine hydroxymethyltransferase to serine

was is taken up? what is removed?