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Biochemistry > Semester 2: Amino acids and nucleotide metabolism > Flashcards

Semester 2: Amino acids and nucleotide metabolism Flashcards

1
Q

What does a negative nitrogen balance mean?

A
  • Excreted nitrogen > ingested nitrogen (starvation, diseases)
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2
Q

What does a positive nitrogen balance mean?

A
  • Ingested nitrogen > excreted nitrogen (children, pregnancy)
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3
Q

What is transamination?

A

A reversible exchange of an amino- and an oxo group (alpha-AA –> alpha-keto acid)

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4
Q

ALAT

A
  • Ala aminotransferase
  • Ala -> Pyruvate
  • Alpha-KG -> Glutamate
  • PLP prostetic group
  • Liver
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5
Q

ASAT

A
  • Asp aminotransferase
  • Oxa -> Asp
  • Alpha-KG -> Glutamate
  • PLP prostetic group
  • Heart/muscle
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6
Q

GLUD1

A
  • Glutamate dehydrogenase isoenzyme used in oxidative deamintaion
  • Found in liver, kidney and other tissues
  • Allosterically inhibited by: ATP, GTP, NADH
  • Activated by ADP
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7
Q

GLUD2

A
  • Glutamate dehydrogenase isoenzyme used in oxidative deamintaion
  • Found in brain, testis
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8
Q

What is the purpose of urea cycle?

A

Excretion of amino group and free ammonia

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9
Q

Allosteric activation of CP synthetase

A

Presence of N-acetyl-glu (from acetyl-CoA and glutamate)

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10
Q

Hyperammonemia type 1

A

alpha-KG is used for removal of ammonia, therefore the citrate cycle is depleted: Low ATP level -> coma
- Type 1: CP synthetase I deficiency -> Arg supplementation

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11
Q

Hyperammonemia type 2

A
  • Ornithine transcarbamoylase def. –> mental retardation
  • Arginosuccinate synthetase def. -> citrullinemia (Arg supplement)
  • Arginosuccinate lyase def. -> arginosuccinate secretion (Arg supplement)
  • Arginase def. -> high Arg level (Arg-free diet)
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12
Q

Succinyl-CoA forming AAs

A

Ser, Cys, Thr, Val, Ile

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13
Q

Pyruvate forming AAs

A

Ala, Ser, Gly

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14
Q

Alpha-ketoglutarate forming AAs

A

Pro, Glu, Gln, Arg, (orn)

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15
Q

Amino acids involved with H4F derivatives

A

His, Trp, Ser, Gly

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16
Q

Glutamate dehydrogenase reaction

A
  • alpha-KG + NH4 + NADPH –> Glu + NADP + H2O

- Glu + NADP + H2O –> alpha-KG + NADPH + NH4

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17
Q

Carbamoyl p synthetase I

A

NH4 + Bicarbonate + 2ATP -> Carbamoyl-P + 2ADP+Pi

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18
Q

Ornithine transcarbamoylase

A

Carbamoyl-p + Ornithine -> Citrulline

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19
Q

Arginosuccinate synthetase

A

Citrulline + Asp + ATP -> Arginosuccinate + AMP+PPi

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20
Q

Arginosuccinate lyase

A

Arginosuccinate -> Arg

+Fumarate

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21
Q

Arginase

A

Arg -> Urea (+ornithine)

22
Q

Serine dehydratase

A

Ser -> aminoacrylate -> pyruvate + NH4

- Coenzyme: PLP

23
Q

Thr dehydratase

A

Thr -> alpha-ketobutyrate

- Coenzyme: PLP

24
Q

Gln synthetase

A

Glu + NH4 + ATP -> Gln + ADP+Pi

25
Q

Asn synthetase

A

Gln + Asp + ATP -> Glu + Asn + AMP+PPi

26
Q

Folate consist of

A

Pterin, para-aminobenzoic acid, glutamic acid

27
Q

DHFR

A

H2F + NADPH -> H4F + NADP

28
Q

MTHFR

A

CH2-H4F + NADH -> CH3-H4F + NAD

29
Q

Homocysteine methyltransferase

A
  • CH3-H4F -> H4F
  • HomoCys -> Met
  • Prostethic group: B12
30
Q

Met adenosyl transferase

A

Met + ATP -> SAM + PPi+Pi

31
Q

SAM dependent methylase

A

SAM -> SAH + CH3

32
Q

SAH hydrolase

A

SAH -> HomoCys + adenosine

33
Q

Cystationine synthase

A
  • PLP + Vit B6

- Ser + Met -> custathione -> Cys + alpha-ketobutyrate

34
Q

Alpha-ketobutyrate DEH complex

A

Alpha-ketobutyrate + NAD -> propionyl-CoA + NADH

35
Q

Propionyl-CoA carboxylase

A
  • Biotin

- Propionyl-CoA + ATP -> Methylmalonyl-CoA + ADP+Pi

36
Q

Methylmalonyl CoA racemase + methylmalonyl-CoA mutase

A
  • B12

Methylmalonyl CoA -> Succinyl CoA

37
Q

Maple syrup urine disease

A

Deficiency of BCKD (branched chain alpha-ketoacid dehydrogenase)
- ketoacidosis, mental retardation

38
Q

Asparaginase

A

Asn -> Asp + NH4+

39
Q

Glutamate decarboxylase

A

Glu -> GABA + CO2

40
Q

PRPP synthetase

A

R-5-P + ATP -> PRPP + ADP

41
Q

AMP deaminase

A

AMP + H2O -> IMP + NH4+

42
Q

Adenylosuccinate synthase

A

IMP + Asp + GTP -> Adenylosuccinate + GDP + Pi

43
Q

IMP dehydrogenase

A

IMP + H2O + NAD -> XMP + NADH

44
Q

GMP synthase

A

XMP + Gln + ATP -> GMP + ADP+Pi + Glu

45
Q

Purine salvage reaction enzymes

A
  • Adenine phsophoribosyl transferase (adenine->AMP, PRPP->PPi)
  • Hypoxanthine guanine phosphoribosyl transferase
46
Q

Catabolism of purine nucleotides, enzymes

A
  • 5´nucleotidase (AMP-> adenosine)

- Purine nucleoside phosphorylase

47
Q

Hyperuricemia (gout)

A

Causes:
- PRPP overproduction (PRPP synthase mut.)
- Overproduction of R-5-P
- Absence of purine salvage reactions (eg. HPRT def.)
- Low ATP level, disturbed ATP metabolism
Medication: Allopurinol

48
Q

Orotic aciduria

A

Causes:

  • UMP-S def.
  • Inhib. of UMP-S
  • Ornithine transcarbamoylase def.
49
Q

Pyrimidine salvage reaction enzyme

A

Uridine kinase, cytidine kinase

50
Q

Thymidylate synthase

A

dUMP -> dTMP

- Inhibited by 5F-dUMP (colon cancer)

Biochemistry (9 decks)

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