MCM-Amino Acid Metabolism Flashcards

1
Q

What Supplies the Amino Acids

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

What Depletes Amino Acids

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

What is Hartnup Disease

A

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

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4
Q

What is an exopeptidase

A

Enzyme that cuts terminal ends of a protein

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5
Q

What are the two exopeptidase’s

A

Aminopeptidase; cuts N terminal Carboxypeptidase; cuts C terminal

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6
Q

What is an Endopeptidase

A

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

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7
Q

What degrades Proteins

A

Lysosomes

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8
Q

When are the proteolytic enzymes of the Lysosome active

A

At 5 pH inside the Lysosome

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9
Q

When are the proteolytic enzymes of Lysosome inactive

A

At 7 pH in the cytoplasm

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10
Q

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

A

Non-selective; Macroautophagy Microautophagy Chaperone-mediated autophagy

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11
Q

Is Proteasomal Degradation Selective or non-selective

A

Selective

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12
Q

How is the Proteasomal Degradation Selective

A

Via Ubiqiunation of the selected proteins

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13
Q

What are the two processes of Intracellular Proteolytic Control

A

Lysosomal Degredation and Proteasomal Degradation

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14
Q

Example of Extracellular Proteolytic Control

A

Zymogens; ex. pepsinogen, trypsinogen, etc

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15
Q

Essential Amino Acids

A

P V T T I M H A L L

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16
Q

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

A

Pyruvate

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17
Q

Where does Leucine enter the TCA Cycle

A

Acetyl-CoA

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18
Q

Where does Glutamate enter the TCA Cycle

A

Alpha-Ketoglutarate

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19
Q

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

A

Glutamate

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20
Q

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

A

Succinyl-CoA

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21
Q

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

A

Tyrosin

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22
Q

Where does Aspartate enter the TCA Cycle

A

Oxaloacetate

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23
Q

What does Asparagine get converted into to enter the TCA Cycle

A

Aspartate

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24
Q

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

A

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

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25
Q

What is a transamination reaction do

A

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

26
Q

Glutamate
converts to ______

Aspartate
Converts to ______

Which enzyme is used:

A

Alpha-Ketoglutarate

Oxaloacetate

Aspartate Aminotransferase

27
Q

Alanine
converts to _____

Glutamate
Converts to _____

This reaction uses the Enzyme
________

A

Pyruvate

Alpha-Ketoglutarate

Alanine Transferase

28
Q

Glutamine
Converts to _____

H2O
Converts to ______

This reaction uses the enzyme Glutamine Aminohydralase

A

Glutamate

NH3

29
Q

What co-enzyme do Transaminases require

A

pyridoxyl-5’-phosphate
Vitamin B6

30
Q

How does amonia leave the brain

A

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
Q

Where are the BCAA’s degraded in the body

A

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

32
Q

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

A

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

33
Q

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

A

Cystinuria

34
Q

What can a B6 deficiency lead to

A

Cystathionine ß-Synthase not working; required co-enzyme

leading to Cystinuria

35
Q

How do Hyperhomocysteinemia and Homocystinuria Present

A

Eye lens dislocation, osteoporsis, and mental retardation

(Eye, Skeletal, CNS)

36
Q

Hyperhomocysteinemia is a risk factor for what disease

A

Athersclerotic heart disease and stroke

37
Q

During prolonged exercise and fasting what do muscles use as fuel

A

BCAA

38
Q

What defective enzyme causes Maple Syrup Disease

A

A deficient
Branched Chain Alpha-Ketoacid Dehydrogenase Complex

39
Q

What is Phenylketonuria

A

Defect in Phenylalanine Hydrolase

40
Q

What happens in Phenylalanine in Phenylketonuria

A

Phenylalanine is converted to Phenylpyruvate and then to Phenylactate and Phenylacetate

41
Q

What do Phenylactate and Phenylacetate do to negatively impact the body

A

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

42
Q

How to treat Phenylketonuria

A

DIetary limitiation of Phenylalanine

43
Q

What are some of Tryptophan’s derivatives

A

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

44
Q

What are some Tyrosine derivatives

A

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

45
Q

What are some Glutamate Derviatives

A

Gama-Amino butyric Acid (GABA)

46
Q

What are some Serine Derivatives

A

Acetylcholine

47
Q

What defective enzyme inhibits Tyrosine -> Dopamine

A

Tyrosine Hydroxylase

48
Q

What is Albinism caused by

A

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

49
Q

What can carcinoid tumors occur

A

Overproduce serotonin

50
Q

How does the brain remove NH4+ (Nitrogen)

A

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
Q

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

A

Muscle

52
Q

Urea Cycle Function

A

Get rid of Nitrogen

53
Q

Defective Ornithine Transcarbamoylase Causes What

A

Hyperammonemia

54
Q

What does Hyperammonemia Cause

A

Build up of NH3;
NH3 is toxic to the cell

55
Q

Consequences of NH3 Build up

A

pH Imbalance; swelling of astrocytes —> cerebral edema

Glutamate Depletion —> GABA depletion

Build up of Ammonia also causes Mitochondrial Disfunction

56
Q

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

A

Tyrosine;

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

57
Q

What does a high protein diet increase the output of

A

Urea

58
Q

What three amino acids make Creatine

A
  1. Arginine
  2. Glycine
  3. Methionine

Arginine and Methionine are essential Amino Acids

59
Q

Where are Ornithine and Citrulline located

A

In the Mitochondria

60
Q

What happens to a Carbamoyl Phosphate in the Urea cycle

A

Gives its NH group to Ornithine; making Citrulline