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Biochem > Nitrogen Metab and Integration of Metabolism > Flashcards

Nitrogen Metab and Integration of Metabolism Flashcards

1
Q

How much is the protein turnover per day

A

300-400g

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2
Q

Catabolism of amino acids turns them into this final product

A

A- keto acid

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3
Q

The first phase of amino acid catabolism involves transferring their amino group to a-ketoglutarate forming this amino acid

A

Glutamate

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4
Q

Enzyme in the transamination step of the 1st phase of amino acid catabolism

A

Aminotransferase

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5
Q

Coenzyme of aminotransferase in Transamination

A

B6 pyridoxal phosphate

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6
Q

In the 2nd step of the 1st phase of AA catabolism, glutamate is oxidatively deaminated to release free ammonia in which organs

A

Liver and kidney

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7
Q

What is the enzyme involved in the oxidative deamination step of AA catabolism?

A

Glutamate dehydrogenase

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8
Q

Transport of ammonia to peripheral tissues is through this amino acid

A

Glutamine through glutamine synthase

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9
Q

Glutamine can also be transported in the blood and deaminated to release ammonia in these organs

A

Liver and kidney through glutaminase

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10
Q

Exceptions to transamination of amino acids to glutamate

A

Lysine
Threonine
Proline
Hydroxyproline

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11
Q

Transport of ammonia in the muscle is possible when pyruvate is transaminated to what amino acid

A

Alanine

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12
Q

Excretion of excess nitrogen in telostean fish which excrete highly toxic ammonia

A

Ammonotelic

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13
Q

Excess nitrogen excretion in birds which secrete uric acid as semisolid Guano

A

Uricotelic

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14
Q

Excess nitrogen excretion in land animals who excrete it as urea

A

Ureotelic

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15
Q

In what organ does the urea cycle occur?

A

Liver only

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16
Q

Where in the cell does urea cycle occur?

A

Both mitochondria and cytosol

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17
Q

Substrates of Urea cycle

A

NH3
Aspartate
CO2

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18
Q

What is the rate limiting step of urea cycle?

A

NH3 + CO2 to carbamoyl phosphate through carbamoyl phosphate synthetase 1

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19
Q

Allosteric activator of carbamoyl phosphate synthetase 1

A

N- acetylglutamate

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20
Q

Most common enzyme deficiency in Urea cycle

A

Ornithine transcarbamoylase

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21
Q

Link of urea cycle to Krebs cycle

A

Fumarate

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22
Q

1 mol of urea needs how many ATP?

A

3 mol of ATP

but 4 high energy bonds (AMP)

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23
Q

Urea can be excreted through

A

Urine

Intestines where bacteria converts it to CO2 and NH3

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24
Q

This disease has a direct neurotoxic effect on the CNS

Tremors, slurring of speech, vomiting, BOV, cerebral edema

A

Hyperammonemia

NH3–a ketoglutarate–glutamate (excitatory)

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25
Q

Most severe form of hereditary hyperammonemia

A

Carbamoyl phosphate synthetase 1 deficiency

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26
Q

Treatment for hereditary hyperammonemia that binds to glutamine and excreted as phenylglutamine

A

Phenylbutyrate

27
Q

Purely ketogenic amino acids (may be converted to acetyl CoA of acetoacetyl CoA)

A

Ketong-genic

Lysine
Leucine

28
Q

Amino acids that are both ketogenic and glucogenic

A

Try Philippine Islands TY

Tryptophan
Phenylalanine
Isoleucine
Tyrosine

29
Q

NO, creatine and polyamines is a derivative of which amino acid

A

Argggh NO Creating here

Arginine

30
Q

Coenzyme A, taurine and glutathione is a derivative of which amino acid

A

TAU pa RINE ba si Christeine, ang cO-A ng puti kaka-Gluta

Taurine
Coenzyme A
Glutathione

31
Q

Products of this amino acid include heme, purines, creatine, glutathione

A

Glycine

32
Q

Thymine, purine and sphingosine comes from this amino acid

A

Serine

33
Q

Cofactor of phenylalanine hydroxylase that may be deficient in phenylketonuria

A

Tetrahydrobiopterin

34
Q

This phenylketone gives the characteristic odor of urine in phenylketonuria

A

Phenylacetate

35
Q

Congenital deficiency in homogentisate oxidase that leads to homogentisic acid accumulation

Urine turns black upon standing, arthralgia, dark connective tissue

A

Alkaptonuria

36
Q

Homocystinuria happens due to defect in the degradation of this amino acid

A

Methionine

37
Q

Ectopis lentis (downward)
Osteoporosis
MR
thrombi formation, MI, stroke in children and adults

A

Homocystinuria

38
Q

Treatment for homocystinuria

A

Vitamin B6 B12 and folate

39
Q

Cystinuria is an inherited defect of renal tubular amino acid transporter for which amino acids in the PCT of the kidneys

A 
COLA
Cystine
Ornithine
Lysine
Arginine
40
Q

Cystinuria can precipitate formation of ______ in the kidney

A

Staghorn calculi

41
Q

This drug is used to treat cystinuria to alkalinize the urine

A

Acetazolamide

42
Q

Methylmalonyl CoA mutase deficiency can cause a defect in the conversion of methylmalonyl to succinyl CoA

Stroke, seizures, enceph at 1 mo to 1 yr old
And involves metabolism of these amino acids

A 
TIM V
Threonine 
Isoleucine
Methionine
Valine
43
Q

Maple syrup urine disease is due to blocked degradation of branched chain amino acids because of deficiency in this enzyme

A

A-ketoacid dehydrogenase

44
Q

These are cyclic compounds formed by the linkage of 4 pyrrole rings through methyne bridges

A

Porphyrins

45
Q

What is the rate limiting step of heme synthesis

A

Formation of d-ALA from glycine and succinyl CoA through ALA synthase

46
Q

Cofactor of ALA synthase in the rate limiting step of heme synthesis

A

Pyridoxine

47
Q

Formation of porphobilinogen is inhibited by

A

Heavy metal ions that replace zinc

48
Q

What enzyme catalyzes the formation of heme from protoporphyrin IX?

A

Ferrochelatase

49
Q

In heme degradation, bilirubin is conjugated to 2 molecules of glucoronic acid for excretion. This enzyme is deficient in Crigler Najjar and Gilbert syndrome

A

Bilirubin glucuronyltransferase

50
Q

Bilirubin in stool is called

A

Stercobilin

51
Q

Bilirubin in urine is called

A

Urobilin

52
Q

Bilirubin in the intestines is called

A

Uronilinogen

53
Q

Enzymes in heme synthesis inhibited by lead

A

ALA dehyratase

Ferrochelatase

54
Q

Most common form of porphyria

A

Porphyria cutanea tarda

55
Q

Porphyria that occurs before the formation of ring leads to accumulation of ALA and PBG and lead to what symptom

A

Neuropsychiatric

56
Q

Accumulation of porphyrinogens in the skin can undergo spontaneous oxidation to porphyrins and cause this symptom

A

Photosensitivity

57
Q

Direct or Indirect hyperbilirubinemia?

Dubin- Johnson
Rotor

A

Direct

58
Q

Crigler Najjar and Gilbert syndrome can cause what type of hyperbiliruninemia

A

Indirect

59
Q

Epinephrine, cortisol and GH has similar effects to what hormone

A

Glucagon

60
Q

Principal site of metabolism of branched chain amino acids

A

Skeletal muscle

61
Q

Energy store used for sprinting

A

Creatine phosphate

62
Q

Energy store used in marathons

A

Glycogen and fatty acids

63
Q

In fasting state, glycogen is used as fuel for how many hours into the fasting?

A

18-24 hours

64
Q

Peak of Ketone bodies being used as fuel in starvation

A

10 days

Biochem (7 decks)

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