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Block 5 > Random-ass MBOD > Flashcards

Random-ass MBOD Flashcards

1
Q

Name the 2 ketogenic AA’s

A

Leu, Lys

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2
Q

Name the amino acids that are both keto and glycogenic?

A

Phe
Tyr
Trp
Ile

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3
Q

The body doesn’t store AA’s. Where does it put them then?

A

Muscle protein

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4
Q

When you are not ingesting food, the body still maintains a normal concentration of free AA’s in the blood. Where do these AA’s come from?

A

THe breakdown of muscle proteins.

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5
Q

In order to use free AA’s, what must be done first? What problem results?

A

Deamination of free AA’s must occur before the body can use them. This creates a buildup of nitrogenous waste in the blood.

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6
Q

What effect do glucocorticoids and thyroid hormones have on the breakdown of proteins>

A

They increase protein breakdown.

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7
Q

REMEMBER: Insulin is the ANABOLIC hormone. Glucagon is CATABOLIC

A

Yeah.

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8
Q

Name some proteins with high turnover rates:

A

Heme, muscle protein, digestive enzymes

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9
Q

What AA is only essential during development and growth, but nonessential in adulthood?

A

Arginine

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10
Q

What non-essential AA is derives from Phe?

A

Tyr

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11
Q

WHat nonessential AA is derives from Met?

A

Cys

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12
Q

How many essential AA’s are there? Name them.

A
  1. PVT TIM HALL
Phe
Val
Thr
Trp
Ile
Met
His
Lys
Leu
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13
Q

The majority of protein degradation that takes place in the body is of endogenous protein or dietary protein?

A

Endogenous.

Dietary protein is broken down and used for synths of new proteins.

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14
Q

Dx this:

  • muscle wasting
  • decreased albumin levels
  • distended abdomen
  • child
  • non-varied diet
A

Kwashiorkor- the absence of dietary essential amino acids

No ability to synthesize pancreatic enzymes and new intestinal cells.

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15
Q

HOw would you treat Kwashiorkor?

A

Add Pancreatic protease to the diet

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16
Q

In muscle, as proteins are broken down, they are trans/deaminated. What AA does the muscle export and what is its purpose?

A

NH3 from all the other AA’s in muscle are funneled to ALANINE for transport to the liver. Rids the muscle of excess nitrogenous waste.

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17
Q

Name the 3 ways AA’s are transported.

A

Na+ dependent carriers
Facilitated Transporter
Gamma - glutamyl cycle

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18
Q

What tissues use the gamma-glutamyl cycle for AA import?

A

Intestine and Kidney

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19
Q

What substance is necessary for the gamma-glutamyl cycle to work?

A

GSH - Glutathione

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20
Q

What 2 diseases of AA transport defects produce hyperaminoacidUREA?

A

Hartnup Disease (Neutral AA Urea)

Cystinuria

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21
Q

What’s the main treatment for Hartnup Disease (Neutral Amino Acid Urea)

A

Niacin and HIGH protein diet

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22
Q

What is the defect in Hartnup disease>

A

The intestine and kidney can’t absorb NEUTRAL and AROMATIC AA’s.

Remember: BOTH INTESTINE AND KIDNEYS INVOLVED

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23
Q

What essential substance is deficient if a person has Hartnup disease?

A

No tryptophan (essential AA), so no NIACIN, which is necessary for Nad+/ NADH

24
Q

What is the defect involved with Cystinuria?

A

KIDNEY ONLY: Kidney can’t absorb cysteine and basic amino acids

Cystine and basic AA’s lost in the urine

25
Q

Do you have an AA deficiency in Cystinuria?

A

No. Cysteine and basic AA’s are non-essential so no deficiency will be noted.

26
Q

What is the worst symptom of Cystinuria?

A

Due to increased amount of Cysteine in the urine, kidney stones form. Cysteine is INSOLUBLE in urine.

27
Q

What are the 2 substances that can cause kidney stones (that we have learned about)?

A

Cystine and Oxalate ?

28
Q

How do you treat Cystinuria?

A

Goal is to increase Cystine solubility.

  • Maintain large urine volume
  • Neutral or basic pH of urine
  • PENICILLAMINE (increases solubility of cystine)
29
Q

Do patients with Hartnup (Neutral AA Urea) or Cystinurea have hyperaminoacidemia?

A

No

30
Q

What are the 2 ways to remove alpha amino groups from an amino acid?

A

Transamination (most common) and deamination

31
Q

The a-ketoacid of Alanine is……

A

Pyruvate

32
Q

The a-ketoacid of Aspartate is……

A

Oxaloacetate

33
Q

What is the only amino acid that can enter the Urea cycle? (Hint: it donates an NH3 to urea)

A

Aspartate

34
Q

The a-ketoacid of glutamate is …..

A

a-ketoglutarate

35
Q

Name the 2 most important transaminases.

A

(ALT) Alanine Amino-transferase

(AST)Aspartate Amino-transferase

36
Q

Presence of both ALT and AST in serum is indicative of…..

A

Liver damage

37
Q

Where will you find the ALT enzyme?

A

IN the mitochondria of liver and cytosol of muscle

38
Q

WHere will you find the AST enzyme?

A

Only in the liver

39
Q

Aminotransferase enzymes require what cofactor?

A

PLP - Piroxidal 5” Phosphate

40
Q

Once the aminotransferases have created glutamate, what deaminase removes the NH3 from it?

A

Glutamate Dehydrogenase -> a-ketoglutarate + NH3 + NADPH

41
Q

What effect does Vitamin B deficiency have on transaminase reactions?

A

Inhibits. Need Vit B6 to synthesize PLP, which is necessary for transaminase activity.

42
Q

Some AA’s are specifically deaminated by certain enzymes. Asn and Gln are examples. What are they deaminated by?

A

Asparaginase and Glutaminase to form Aspartate and Glutamate, which are then deaminated by ALT in liver, and glutamate dehydrogenase.

43
Q

Serine and Threonine are directly de-aminated by _____, which requires ______ to function.

A

Ser and The Dehydrogenase- PLP dependent!

44
Q

At physiological pH, what form is nitrogen in? (NH3, NH4+)

Which form can cross cell membranes and into kidney tubules?

A

NH4+ is most common at physiological pH

NH3 is able to be secreted

45
Q

How does ammonia regulate the pH of urine?

A

It is secreted into the kidney tubules as NH3. ONce in the urine, it binds free H+ atoms to form NH4+. This form can no longer cross the membrane

46
Q

What species within the era cycle carry the 2 nitrogens to donate to urea?

A

Citruline and Aspartate

47
Q

Carbamoyl Phosphate uses what enzyme?

A

CPS1 (carbamoyl -Pi synthase 1)

48
Q

CPS1 relies on what coenzyme?

A

NAG / NAG synthase

49
Q

Deficiency in NAG, NAGS, or CPS1 causes what?

A

Type 1 Hyperammonemia - Urea cycle shuts down

50
Q

How do you cure type 1 hyperammonemia?

A

Benzoate and Phenylacetate to rid the blood of Glu and Gln. The body is then forced to use the excess NH4+ for essential AA synthesis.

51
Q

How do you diagnose an OTC deficiency?

A

OTC is Ornithine transcarbamylase, which catalyzes the formation of citrulline. So no citruline, and OROTIC ACID is present.

52
Q

What other pathway is orotic acid involved in?

A

Pyrimidine synthesis. So turn urea cycle off, turn on pyrimidine synthesis.

53
Q

How does excess urea affect the brain?

A

The body stores excess NH4+ as Glu and Gln. The order goes a-ketoglutarate to glutamate, to glutamine. Eventually, all the glutamate is used up and a-ketoglutarate is used to form more. This depletes the TCA cycle= cell death.

Also, no more glutamate = no more GABA

Increased osmotic pressure = edema

54
Q

Mutations in both CPS1 and OTC cause…..

A

Hyperammonemia, which can be treated with benzoate and phenyl acetate.

55
Q

Arginosuccinase deficiency can be partially bypassed by:

A
  • Restricting protein intake

- ARGININE SUPPLEMENTS

Block 5 (10 decks)

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