MCM-Amino Acid Metabolism

Question 1

What Supplies the Amino Acids

Answer 1

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

Question 2

What Depletes Amino Acids

Answer 2

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

Question 3

What is Hartnup Disease

Answer 3

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

Question 4

What is an exopeptidase

Answer 4

Enzyme that cuts terminal ends of a protein

Question 5

What are the two exopeptidase’s

Answer 5

Aminopeptidase; cuts N terminal Carboxypeptidase; cuts C terminal

Question 6

What is an Endopeptidase

Answer 6

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

Question 7

What degrades Proteins

Answer 7

Lysosomes

Question 8

When are the proteolytic enzymes of the Lysosome active

Answer 8

At 5 pH inside the Lysosome

Question 9

When are the proteolytic enzymes of Lysosome inactive

Answer 9

At 7 pH in the cytoplasm

Question 10

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

Answer 10

Non-selective; Macroautophagy Microautophagy Chaperone-mediated autophagy

Question 11

Is Proteasomal Degradation Selective or non-selective

Answer 11

Selective

Question 12

How is the Proteasomal Degradation Selective

Answer 12

Via Ubiqiunation of the selected proteins

Question 13

What are the two processes of Intracellular Proteolytic Control

Answer 13

Lysosomal Degredation and Proteasomal Degradation

Question 14

Example of Extracellular Proteolytic Control

Answer 14

Zymogens; ex. pepsinogen, trypsinogen, etc

Question 15

Essential Amino Acids

Answer 15

P V T T I M H A L L

Question 16

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

Answer 16

Pyruvate

Question 17

Where does Leucine enter the TCA Cycle

Answer 17

Acetyl-CoA

Question 18

Where does Glutamate enter the TCA Cycle

Answer 18

Alpha-Ketoglutarate

Question 19

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

Answer 19

Glutamate

Question 20

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

Answer 20

Succinyl-CoA

Question 21

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

Answer 21

Tyrosin

Question 22

Where does Aspartate enter the TCA Cycle

Answer 22

Oxaloacetate

Question 23

What does Asparagine get converted into to enter the TCA Cycle

Answer 23

Aspartate

Question 24

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

Answer 24

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

Question 25

What is a transamination reaction do

Answer 25

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

Question 26

Glutamate converts to \_\_\_\_\_\_ Aspartate Converts to \_\_\_\_\_\_ Which enzyme is used:

Answer 26

Alpha-Ketoglutarate Oxaloacetate Aspartate Aminotransferase

Question 27

Alanine converts to \_\_\_\_\_ Glutamate Converts to \_\_\_\_\_ This reaction uses the Enzyme \_\_\_\_\_\_\_\_

Answer 27

Pyruvate Alpha-Ketoglutarate Alanine Transferase

Question 28

Glutamine Converts to \_\_\_\_\_ H₂O Converts to \_\_\_\_\_\_ This reaction uses the enzyme Glutamine Aminohydralase

Answer 28

Glutamate NH₃

Question 29

What co-enzyme do Transaminases require

Answer 29

pyridoxyl-5'-phosphate Vitamin B₆

Question 30

How does amonia leave the brain

Answer 30

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

Question 31

Where are the BCAA's degraded in the body

Answer 31

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

Question 32

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

Answer 32

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

Question 33

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

Answer 33

Cystinuria

Question 34

What can a B₆ deficiency lead to

Answer 34

Cystathionine ß-Synthase not working; required co-enzyme leading to Cystinuria

Question 35

How do Hyperhomocysteinemia and Homocystinuria Present

Answer 35

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

Question 36

Hyperhomocysteinemia is a risk factor for what disease

Answer 36

Athersclerotic heart disease and stroke

Question 37

During prolonged exercise and fasting what do muscles use as fuel

Answer 37

BCAA

Question 38

What defective enzyme causes Maple Syrup Disease

Answer 38

A deficient Branched Chain Alpha-Ketoacid Dehydrogenase Complex

Question 39

What is Phenylketonuria

Answer 39

Defect in Phenylalanine Hydrolase

Question 40

What happens in Phenylalanine in Phenylketonuria

Answer 40

Phenylalanine is converted to Phenylpyruvate and then to Phenylactate and Phenylacetate

Question 41

What do Phenylactate and Phenylacetate do to negatively impact the body

Answer 41

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

Question 42

How to treat Phenylketonuria

Answer 42

DIetary limitiation of Phenylalanine

Question 43

What are some of Tryptophan's derivatives

Answer 43

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

Question 44

What are some Tyrosine derivatives

Answer 44

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

Question 45

What are some Glutamate Derviatives

Answer 45

Gama-Amino butyric Acid (GABA)

Question 46

What are some Serine Derivatives

Answer 46

Acetylcholine

Question 47

What defective enzyme inhibits Tyrosine -\> Dopamine

Answer 47

Tyrosine Hydroxylase

Question 48

What is Albinism caused by

Answer 48

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

Question 49

What can carcinoid tumors occur

Answer 49

Overproduce serotonin

Question 50

How does the brain remove NH₄⁺ (Nitrogen)

Answer 50

Alpha-Ketoglutarate + NH₄⁺ + NADPH ---\> Glutamate; Glutamate + NH₄⁺ + ATP ---\> Glutamine + ADP + P_i Glutamine is transferred to the liver and Glutamate is remade and Ammonium is excreted

Question 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

Answer 51

Muscle

Question 52

Urea Cycle Function

Answer 52

Get rid of Nitrogen

Question 53

Defective Ornithine Transcarbamoylase Causes What

Answer 53

Hyperammonemia

Question 54

What does Hyperammonemia Cause

Answer 54

Build up of NH_3; NH₃ is toxic to the cell

Question 55

Consequences of NH₃ Build up

Answer 55

pH Imbalance; swelling of astrocytes ---\> cerebral edema Glutamate Depletion ---\> GABA depletion Build up of Ammonia also causes Mitochondrial Disfunction

Question 56

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

Answer 56

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

Question 57

What does a high protein diet increase the output of

Answer 57

Urea

Question 58

What three amino acids make Creatine

Answer 58

1. Arginine 2. Glycine 3. Methionine Arginine and Methionine are essential Amino Acids

Question 59

Where are Ornithine and Citrulline located

Answer 59

In the Mitochondria

Question 60

What happens to a Carbamoyl Phosphate in the Urea cycle

Answer 60

Gives its NH group to Ornithine; making Citrulline