Lecture 4: Amino Acid Degredation Flashcards

1
Q

Products from removal of amino group from AA

A

Alpha-keto acid and Ammonia

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2
Q

Transamination

A

Transferring alpha amino group to alpha ketoglutarate (most common N acceptor)

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3
Q

Transamination product

A

Glutamate

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4
Q

Transamination enzyme

A

Aminotransferase

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5
Q

Deamination

A

Releases ammonia from glutamate to liver

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6
Q

Deamination Enzyme

A

Glutamate Dehydrogenase

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7
Q

Urea formation rate limiting step

A

Carbamoyl Phosphate Synthetase 1

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8
Q

Urea formation in Liver equation

A

CO2 + Free Ammonia + H2O ——> Carbamoyl Phosphate

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9
Q

Where do the nitrogen’s come from in urea production?

A

Aspartate and ammonia

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10
Q

Step 2 of Urea production in liver

A

Carbamoyl Phosphate ——> L-Citruline

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11
Q

Final step of urea production

A

L-Arginine ——> L-Ornithine

Releases Urea

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12
Q

Arginase

A

L-Arginine ——> L-Ornithine

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13
Q

What enzyme breaks down extra urea in the intestine?

A

Bacterial Urease

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14
Q

What do Glucogenic AA’s produce?

A

Pyruvate

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15
Q

What do Ketogenic AA’s produce?

A

Acetoacetate (or precursors)

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16
Q

Phenylketonuria (PKU)

A

Excessive Phenylalanine

Not enough Phenylalanine Hydroxylase

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17
Q

Phenylalanine ——> Tyrosine

A

Phenylalanine Hydroxylase

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18
Q

Lack of _____ causes albinism by blocking melanin formation

A

Tyrosinase

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19
Q

_______ Produced in liver and erythrocyte producing cells of bone marrow

A

Porphyrins

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20
Q

ALAS1 inhibited by

A

Heme overpresence (negative feedback loop)

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21
Q

Lead inhibits

A

Aminolevulunic Acid Dehydratase

22
Q

Lead also inhibits

A

Ferrochelatase

23
Q

Heme ——> Biliverdin (step 1 Heme degradation) enzyme is ____

A

Heme Oxygenase

24
Q

Biliverdin to Bilirubin (heme degradation step 2) enzyme is ______

A

Biliverdin Reductase

25
Glucuronic Acid
Solubilizes toxic Bilirubin Jaundice occurs without it
26
Catecholamine starting AA
Tyrosine
27
Tyrosine ——> DOPA enzyme
Tyrosine Hydroxylase
28
NorEpi or Epi ——> VMA by which enzymes?
COMT MAO SAM (donates methyl)
29
Dopamine’s two fates
Norepinephrine HVA (alternative path)
30
Serotonin converted from…
Tryptophan
31
Arginine and/or Glycine form this product
Creatine Phosphate
32
Tyrosinase is the enzyme for what reaction?
Tyrosine ——> Melanin
33
Ribose-5-Phosphate ——> PRPP by which enzyme?
PRPP Synthetase And ATP
34
Ribonucleotide Reductase reaction
AMP ——> dAMP
35
Ribonuclease Reductase inhibited by …
Hydroxyurea
36
Intermediate in Glutamine ———> CMP (Pyrimidine synthesis)
UMP (Glut —> UMP —> CMP)
37
Thymidylate Synthetase
dUMP ——> dTMP Inhibited by 5-fluorouracil
38
5-fluorouracil
Inhibits Thymidylate Synthetase
39
CTP Synthase reaction
UTP ——> CTP
40
Thiamine (B1) Deficiency
Beriberi Wernicke-Korsakoff
41
Riboflavin (B2) Deficiency
Cheilosis - fissures at corner of mouth Glossitis - smooth purple tongue
42
Niacin (B3) Deficiency
Pellagra - skin and GI
43
Pantothenic Acid (B5) Deficiency
No known deficiency
44
Pyridoxine (B6) Deficiency
Only Toxic water soluble vitamin Dermatitis, anemia, depression, convulsions
45
Biotin (B7) Deficiency
No known deficiency
46
Folic Acid (B9) Deficiency
Pregnancy issues Spina Bifida and Anencephaly
47
Cobalamin (B12) Deficiency
Deficient in some vegetarians Pernicious Anemia
48
Ascorbic Acid (C) Deficiency
Scurvy
49
Tocopherol (E) Deficiency
Nerve damage + blood cell breakage
50
Phylloquinone, Menaquinone (K) Deficiency
Hemorrhaging
51
Vitamin A Deficiency
Night Blindness Xeropthalmia - dry eyes Keratomalacia - softening of cornea
52
Vitamin D Deficiency
Rickets Osteoporosis Osteomalacia