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M1 - MCM Exam 1 > Amino Acids Metabolism > Flashcards

Amino Acids Metabolism Flashcards

1
Q

Supplies for the Amino Acid Pool

A
  1. Protein turnover - proteins salvaged and repurposed in the body
  2. Digested food - mostly from meat, veggies, and nuts
  3. De novo synthesis - synthesis of non-essential amino acids
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2
Q

Sources that Deplete the Amino Acid Pool

A
  1. Production of body protein
  2. Synthesis of nitrogen-containing compounds
  3. Degradation of essential amino acids
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3
Q

Proteolytic Enzymes

A

Degradation of proteins for reabsorption

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4
Q

Two Classes of Proteolytic Enzymes

A
  • Exopeptidase - attacks the N or O terminus ends
    • Carboxypeptidase - Attacks and adds H2O at the O end
    • Aminopeptidase - Attacks and adds H2O at the N end
  • Endopeptidase - attacks within the protein at a specific site
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5
Q

Lysosomes

A

Performs autophagy

Contains over 50 proteolytic enzymes

Internal environment within lysosome has a pH of 5

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6
Q

Proteasome

A

A large protein complex within the cells that breaks apart marked proteins

Marker - ubiquinitin

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7
Q

Ubiquitin

A

Marker of proteins that need to be degraded within a cell

Polyubiquitinated

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8
Q

Zymogens

A

The inactive form that most digestive enzymes are secreted

Become active when they get to their target

Ex. Trypsinogen - activated by enterokinase after getting into small intestine lumen - becomes trypsin

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9
Q

Essential Amino Acids

A

PVT TIM HALL

  • Phenylalanine
  • Valine
  • Tryptophan
  • Threonine
  • Isoleucine
  • Methionine
  • Histidine
  • Arginine
  • Leucine
  • Lysine

Occassionally Glutamine - depending on deficiency

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10
Q

Ketogenic Amino Acids

A

Leucine and Lysine

2:5:13

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11
Q

Ketogenic and Glucogenic Amino Acid

A

Isoleucine, Tryptonphan, Phenylalanine, Tyrosine, and Threonine

2:5:13

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12
Q

Glucogenic Amino Acids

A

Valine, Histidine, Methionine, Glycine, Arginine, Alanine, Proline, Aspartic Acid, Serine, Glutamic Acid, Glutamine, Cysteine, Asparagine

2:5:13

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13
Q

Why is the TCA cycle Anaplerotic?

A

Anaplerotic - reactions that supply intermediates for a metabolic pathway

Amino acids supply the TCA cycle with substrates if needed

Pyruvate carboxylation (requires ATP) converts pyruvate to oxaloacetate (OAA)

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14
Q

Reaction that Shuffles Amine groups

A

Transamination

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15
Q

Enzymes that perform transamination

A

Transaminases/Aminotransferase

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16
Q

Transaminase coenzyme

A

PLP (pyridoxyl-5’-phosphate)

Derivative of Vit B6

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17
Q

AST/ALT

A

Aspartate transaminase (AST) Alanine transaminase (ALT)

Used as clinical markers for liver function

18
Q

Amino acids that are metabolized into alpha-ketoglutarate

A

Gln, His, Arg, Pro all converted to Glutamine

Glutamine is then converted into alpha-ketoglutarate

19
Q

Branched Chain Amino Acid Metabolism

A

Valine and Isoleucine - converted to Succinyl-CoA

Leucine - Converted to Acetyl-CoA

If deficiency causes Maple-Syrup urine disease

20
Q

Methionine Metabolism

A

Converted into Succinyl-CoA

Able to produce Cysteine

If issues in metabolism - commonly results in Homocystinuria

21
Q

Threonine Metabolism

A

Converted into Succinyl-CoA

22
Q

What two enzymes are responsible for Homocystinuria?

A

Homocysteine methyltransferase - converts homocysteine back to Methionine

Cystathionine-B-synthase - converts homocysteine into cystathionine (enzymes requires PLP (active form of B6)

23
Q

Hyperhomocysteinemia and Homocystinuria

A

Usually caused by vitamins (B6, B12, or Folic Acid (B9)) or genetic disorder

Risk factor for heart disease and stroke

Treated usually with vitamin suppliments

24
Q

Maple Syrup Urine Disease

A

Deficiency in BCKD (branched-chain alpha-keto acid dehydrogenase complex)

Inability to break down branched-chain amino acids

Presents as branched-chain amino acids in urine which gives urine a sweet smell

High prevalance in Jewish, Amish, and Mennonite populations

25
Q

Deficiency in BCKD

A

Maple Syrup Urine Disease

26
Q

Phenylalanine Metabolism

A

Phenylalanine converted to Tyrosine then to Fumarate

27
Q

Phenylketonuria (PKU)

A

Mutation or disfunction of Phenylalanine hydroylase and inability to convert Phe to Tyr

Results in accumulation of phenylpyruvate and phenyllacetate - elevated levels are toxic to CNS

Musty-odored urine

28
Q

Asparagine Metabolism

A

Asparagine is metabolized into Aspartic Acid then into oxaloacetate

29
Q

What is Aspartic Acid derived from?

A

Asparagine

30
Q

Tryptophan Derivatives

A

NAD+/NADP+ and Serotonin

31
Q

Tyrosine Derivatives

A

T3 (Triiodothyronine), T4 (Thyroxine), Melanin, DOPA, and Oopamine (synthesized into Norepinephrine and Epinephrine)

32
Q

What common diseases are associated with Tryosine derivatives?

A

Grave’s Disease (Hyperthyroidism) or Hypothyroid

Albinism

Parkinsonism

33
Q

Thyroglobulin and Thyroid Hormone

A

Thyroglobulin produces T3 and T4

Uses tyrosine in the binding site

34
Q

Removing Nitrogen in Brain

A

As ammonia

Remove Glutamine and Glutamic acid as ammonia

CO - comes from CO2

1 NH2 - NH4+

NH2 - from aspartate

35
Q

Removal of Nitrogen in Muscles

A

In muscles combines pyruvate and glutamate to make alpha-ketoglutarate and alanine (nitrogen goes on Alanine

They excrete alanine which travels to the liver and the nitrogen is freed into ammonia and enters the urea cycle

36
Q

Transamination vs. Oxidative deamination

A

Transamination move the amino group

Aminotransferase produces ammonia

37
Q

Urea Cycle Common Diseases

A

Hyperammonemia w/ orotic aciduria - malfuction of enzyme in between ornithine and citrulline

Hyperammonemia - where ammonium is unable to be incorporated into carbarnoyl phosphate

Citrullinemia - a build up of citrulline

Argininosuccinate aciduria -

38
Q

Ammonia Toxicity

A

Issue because NH3 is able to cross the blood-brain barrier

Causes pH imbalance and brain swelling

Messes with glutamate dehydrogenase to alpha-ketoglutarate becuase of the abundance of ammonia which stalls TCA cycle

39
Q

Amino acids associated with Phosphocreatine

A

Arg, Gly, and Met

40
Q

Location and Purpose of Phosphocreatine

A

Located in spern, brain and muscles

Used as a storage form of energy

41
Q

What is creatine a biomarker for?

A

Kidney function

42
Q
A

M1 - MCM Exam 1 (7 decks)

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