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MCM > Protein and AA metabolism > Flashcards

Protein and AA metabolism Flashcards

1
Q

What three things supply the AA pool?

A
  • Protein turnover
  • Digested food
  • De novo synthesis
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2
Q

What three things deplete the AA pool?

A
  • Production of body proteins
  • synthesis of nitrogen containing compounds
  • Degradation of **Essential amino acids
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3
Q

What is proteolysis?

A

degradation of proteins for reabsorption

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4
Q

How does an exopeptidase work?

A

Attacks the C and N-terminus (ends)

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5
Q

How does an endopeptidase work?

A

attacks within the protein at a specific site (digests internal peptide bonds.

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6
Q

Define lysosomal/autophagy

A

non-selective intracellular proteolytic control

  • macroautophagy
  • microautophagy
  • chaperone-mediated autophagy (CMA)
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7
Q

Define Proteasomal degredation

A

large proteasome cytoplasmic complexes that cleave polyubiquinated proteins-> ubiquitin pathway

  • selective pathway
  • death tag
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8
Q

What is trypsinogen activated by?

A

enterokinase

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9
Q

How are proteolytic enzymes secreted?

A

They are secreted as needed and as inactive zymogens, which are activated by proteolytic cleavage

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10
Q

What are the 2 ketogenic AAs

A

Leu

Lys

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11
Q

What are the 5 ketogenic and gucogenic AAs

A 
Phe
Ile
Tyr
Thr
Trp
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12
Q

What are the 13 glucogenic AAs

A

Val , His, Arg, Asn, Gln,
Met, Ala, Asp, Glu
Gly, Pro, Ser, Cys

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13
Q

What are ketogenic AAs degrated to?

A

Acetyl CoA or acetoacetate -> precursors for alpha ketoacids, ketone bodies, and fatty acids

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14
Q

What are glucogenic AAs degraded to?

A

Pyruvate or TCA cycle intermediates
-OAA, alpha ketogluterate,
Succinyl CoA, fumarate
(precursors for glucose synthesis)

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15
Q

Essential AAs

A

PVT TIM HALL

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16
Q

What AAs are degraded to alpha-ketogluterate?

A

Gln, His, Arg, Pro, Glu

17
Q

What causes homocystinuria

A

defective enzume cystathionine beta-synthase
or deficiencies of b6, b12,or folic acid
homocystine cannot be converted to cystathionine
*eye, skeletal, CNS, Vascular

18
Q

What are the branched chain amino acids

A

Met, Thr, Val, Ile, Leu –> succinyl CoA or Acetyl CoA

19
Q

What does the body use for fuel during fasting and prolonged exercise?

A

Branched Chain AAs

20
Q

What is maple syrup urine disease?

A

deficient branched chain alpha-keto acid dehydrogenase complex (BCKD) –> LEADS TO BRANCHED CHAIN KETOACIDURIA
-treat with synthetic diet limiting Val Leu Ile

21
Q

What is Leu degraded to

A

Acetyl CoA

22
Q

What is the end product of Phe metabolism

A

fumarate

23
Q

What is the end product of Asn metabolism

A

Oxaloacetate

24
Q

What is the cause of phenylketoneuria

Symptoms and treatment

A

PKU is caused by defects in the activity of phenylalanine hydroxylase (PAH)

25
Q

Instead of the production of tyrosine from phenylalanine, what accumulates in phenylketonuria

A

phe is converted to phenylpyruvate and then to phenyllactate (musty urine) and phenylacetate

26
Q

What do triiodothyronine (T3) and Thyroxine (T4) monitor

A

thyroid disease

27
Q

What enzyme is blocked in albinism

A

tyrosinase defect stops conversion of Tyr to melanin

28
Q

What enzyme is affected in parkinsonism

A

dopa decarboxylase

-negatively affected by Carbidopa

29
Q

What does thyroglobulin produce

A

T3 and T4

30
Q

What do carbimazole and propylthiouracil treat

A

hyperthyroidism by reducing production of T3 and T4

31
Q

Why is NH3 toxic, rather than NH4+

A

NH3 can traverse biological membranes

32
Q

What does a defect in the ornithine transcarbamoylase cause

A

mitochondrial buildup of carbamoyl phosphate, which spills out into the cytoplasm

33
Q

What can cause hyperammonemia

A

a defect of any of the 6 enzyes of the urea cycle (including NAG synthase)

MCM (6 decks)

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