MCM-Amino Acid Metabolism Flashcards Preview

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Flashcards in MCM-Amino Acid Metabolism Deck (60):
1

What Supplies the Amino Acids

1. Protein Turnover 2. Digested food 3. De Novo Synthesis

2

What Depletes Amino Acids

1. Production of Body Protein 2. Synthesis of Nitrogen-Containing Compounds 3. Degradation -> making essential Amino Acids

3

What is Hartnup Disease

A dysfunctional neutral amino acid transporter. Most notably effecting Tryptophan; leading to a deficiency. Presents as Pellagra; since Tryptophan deficiency also means Niacin deficiency

4

What is an exopeptidase

Enzyme that cuts terminal ends of a protein

5

What are the two exopeptidase's

Aminopeptidase; cuts N terminal Carboxypeptidase; cuts C terminal

6

What is an Endopeptidase

Attacks the proteins specific site internally; opposed to the terminal ends

7

What degrades Proteins

Lysosomes

8

When are the proteolytic enzymes of the Lysosome active

At 5 pH inside the Lysosome

9

When are the proteolytic enzymes of Lysosome inactive

At 7 pH in the cytoplasm

10

Is Lysosomal Degradation of enzymes selective or non-selective; and which types are included in lysosomal degradation

Non-selective; Macroautophagy Microautophagy Chaperone-mediated autophagy

11

Is Proteasomal Degradation Selective or non-selective

Selective

12

How is the Proteasomal Degradation Selective

Via Ubiqiunation of the selected proteins

13

What are the two processes of Intracellular Proteolytic Control

Lysosomal Degredation and Proteasomal Degradation

14

Example of Extracellular Proteolytic Control

Zymogens; ex. pepsinogen, trypsinogen, etc

15

Essential Amino Acids

P V T T I M H A L L

16

Where do Cystein, Alanine, and Serine enter the TCA Cycle

Pyruvate

17

Where does Leucine enter the TCA Cycle

Acetyl-CoA

18

Where does Glutamate enter the TCA Cycle

Alpha-Ketoglutarate

19

What does Glutamine, Histidine, Arginine, and Proline get converted to, to enter the TCA Cycle

Glutamate

20

Where does Isoleucine, Valine, Methionine, and Threonine enter the TCA Cycle

Succinyl-CoA

21

What does Phenylalanine get converted to, to enter the TCA Cycle

Tyrosin

22

Where does Aspartate enter the TCA Cycle

Oxaloacetate

23

What does Asparagine get converted into to enter the TCA Cycle

Aspartate

24

What does Pyruvate Carboxylate add to the TCA Cycle via Anaplerotic Reaction 

Oxaloacetate from Pyruvate; only in energy rich conditions or high need for Aspartate and Asparagine

25

What is a transamination reaction do

Amino group; from an amino acid is trasnferred to an Alpha-Ketoacid

26

Glutamate
converts to ______

Aspartate
Converts to ______
 

Which enzyme is used:

Alpha-Ketoglutarate



Oxaloacetate

 

Aspartate Aminotransferase

27

Alanine
converts to _____

Glutamate
Converts to _____

This reaction uses the Enzyme
________

Pyruvate


Alpha-Ketoglutarate

Alanine Transferase

28

Glutamine
Converts to _____

H2O
Converts to ______

This reaction uses the enzyme Glutamine Aminohydralase

 

 

Glutamate


NH3

29

What co-enzyme do Transaminases require

pyridoxyl-5'-phosphate
Vitamin B6
 

30

How does amonia leave the brain

Glutamate to Glutamine
Once Glutamine is made
Glutamine leave the brain and is processed in the liver/kidneys back into Glutamate and Amonia leaves the body via urine

31

Where are the BCAA's degraded in the body

The liver lacks the required enzymes; so they are degraded in the:
Skeletal Muscles
Kidneys
and
Brain

32

What does a faulty Cystothionie ß-synthase cause in the body

Homocysteine builds up in the cell
Leads to a di-sulfide bridge forming between two Homociysteine molecules to form Homocystine

33

When there is a build up of Homecystine in the body what happens

Cystinuria

34

What can a B6 deficiency lead to

Cystathionine ß-Synthase not working; required co-enzyme

leading to Cystinuria

35

How do Hyperhomocysteinemia and Homocystinuria Present

Eye lens dislocation, osteoporsis, and mental retardation
(Eye, Skeletal, CNS)

36

Hyperhomocysteinemia is a risk factor for what disease

Athersclerotic heart disease and stroke

37

During prolonged exercise and fasting what do muscles use as fuel

BCAA

38

What defective enzyme causes Maple Syrup Disease

A deficient
Branched Chain Alpha-Ketoacid Dehydrogenase Complex

39

What is Phenylketonuria

Defect in Phenylalanine Hydrolase

40

What happens in Phenylalanine in Phenylketonuria

Phenylalanine is converted to Phenylpyruvate and then to Phenylactate and Phenylacetate

41

What do Phenylactate and Phenylacetate do to negatively impact the body

Disrupt Neurotransmission and Block Amino Acid Transport in the brain; also impacting myelin formation resulting in severe impairment of brain function

42

How to treat Phenylketonuria

DIetary limitiation of Phenylalanine

43

What are some of Tryptophan's derivatives

Serotonin; which leads to melatonin
Niacin; which leads to NAD(P)+

44

What are some Tyrosine derivatives

Dopamine; which can lead to Norepinephrine and then Epinephrine
Thyroid Hormones
Melanin

45

What are some Glutamate Derviatives

Gama-Amino butyric Acid (GABA)

46

What are some Serine Derivatives

Acetylcholine

47

What defective enzyme inhibits Tyrosine -> Dopamine

Tyrosine Hydroxylase

48

What is Albinism caused by

The lack of melanin; caused by a defect in the enzyme Tyrosinase 

49

What can carcinoid tumors occur

Overproduce serotonin

50

How does the brain remove NH4(Nitrogen)

Alpha-Ketoglutarate + NH4+ + NADPH   --->
Glutamate;

Glutamate + NH4+ + ATP ---> Glutamine + ADP + Pi

Glutamine is transferred to the liver and Glutamate is remade and Ammonium is excreted

51

Pyruvate picks up an Amonia and is converted to Alanine, which goes into the liver and then urea cycle to discard the Amonia from what

Muscle

52

Urea Cycle Function

Get rid of Nitrogen

53

Defective Ornithine Transcarbamoylase Causes What

 

Hyperammonemia

54

What does Hyperammonemia Cause

Build up of NH3;
NH3 is toxic to the cell

55

Consequences of NH3 Build up

pH Imbalance; swelling of astrocytes ---> cerebral edema

Glutamate Depletion ---> GABA depletion

Build up of Ammonia also causes Mitochondrial Disfunction

56

Deficiency of Phenylalanine can lead to a deficiency of what amino acid

Tyrosine;

But like not really since Tyrosine is a non-essential amino acid....but AGBAS wants this so we give it to him

57

What does a high protein diet increase the output of

Urea

58

What three amino acids make Creatine

1. Arginine
2. Glycine
3. Methionine

Arginine and Methionine are essential Amino Acids

59

Where are Ornithine and Citrulline located

In the Mitochondria

60

What happens to a Carbamoyl Phosphate in the Urea cycle

Gives its NH group to Ornithine; making Citrulline