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Biochem Prelim 2nd Sem > Inborn Errors Of Urea Cycle > Flashcards

Inborn Errors Of Urea Cycle Flashcards

0
Q

Urea cycle also called

A

Ornithine cycle

Kreb’s henseleit cycle

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1
Q

Urea

A

Carbamide

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2
Q

Urea cycle consists of five reactions

A

2 mitochondria

3 cytosolic

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3
Q

CO2 + H2O ( bicarbonate and ammonia) to carbamoyl phosphate

A

Carbamoyl phosphate synthetase 1

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4
Q

Carbamoyl phosphate + ornithine

A

Citrulline

Enzyme: ornithine transcarbamoylase

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5
Q

How many ATo used in first reaction of urea cycle?

A

2 ATP

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6
Q

Citrulline to arginosuccinate

A

Arginisuccinate synthase

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7
Q

How many ATP used un 3rd reaction of urea cycle?

A

1ATP

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8
Q

Citrulline reacts with_______ then convert to arginosuccinate with the enzyme ________

A

Aspartic acid

Arginosuccinate synthetase

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9
Q

Fumarate form of urea goes to

A

Krebs cycle

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10
Q

Must be transported back to mitochondria to continue the urea cycle

A

Ornithine

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11
Q

Citrulline reacts to ______ and ysed arginosuccinate synthase

A

Aspartate

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12
Q

Arginosuccinate become ________ and ________ by the used of enzyme __________

A

Arginine and fumarate

Arginosuccinate lyase

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13
Q

Arginine to ornithine

A

Arginase

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14
Q

As by product in 3rd reaction in cytosol produce

A

Urea

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15
Q

AA that nitrogen travels in the blood

A

Alanine

Glutamine

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16
Q

CPS1

A

Urea cycle
Mitochondria
Ammonia
Activate by N-AGA ( N-AcetylGlutamicAcid)

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17
Q

CPS2

A

Pyrimidine
Cytosol
Glutamine
No activator

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18
Q

Deficient enzyme in hyperammonemia type 1

A

Carbamoyl phosphate

19
Q

All disorder in UREA cylce is autosomal except to

A

Hyperammonemia type2

20
Q

Chromosome that affected in CPS deficiency?

A

Chromosome 2q35

21
Q

Urinary orotic acid is usually low or absent

A

Hyperammonemia type 1

22
Q

Deficient enzyme in hyperammonemia type 2

A

Ornithine trans carbomylase

23
Q

Most common form of the urea cycle disorder

24
Q

Walang elevation of glutamine and alanine sa

25
Q

Deficient enzyme in citrullinemia

A

Arginosuccinate synthetase

26
Q

Laboratory finding are similar to those in type2 but increase plasma citrulline mas mataas din ang orotic acid.

A

Citrullinemia

27
Q

Loaction of citrullinemia

A

Chromosome 9q34

28
Q

Enzyme deficient in arginosuccinate aciduria

A

Arginosuccinase

29
Q

Lacation of arginosuccinate aciduria

A

Chromosome 7

30
Q

Enzyme defect in hyoerarginemia

31
Q

Location of hyperarginemia

A

Chromosome 6q23

32
Q

Increase glutamine and alanine but decrease citrulline

A

Hyperammonemia type 1

33
Q

Increase glutamine and alanine

A

Hyperammonemia type 2

34
Q

Increase citrulline

A

Citrullinemia

35
Q

Increase gluatamine, alanine, citrulline and arginosuccinic acid

A

Arginosuccinate acidosis

36
Q

Increase arginine, normal or increase lysine, cysteine and ornithine

37
Q

Decrease or absent of orothic acid

A

Hyperammonemia type 1

38
Q

Increase orothic acid

With gravel stone

A

Hyper ammonemia type 2

39
Q

Increase orothic acid

A

Citrullinemia

Argenemia

40
Q

Carbamoyl phosphate + aspartate

A

Orotic acid

41
Q

No conversion of NH4 to urea

A

HYperammonemia

42
Q

Glutamic acid + ammonia

A

Glutamine

Leads to hyperglutaminemia

43
Q

Ammonia react with alpha ketoglutarate to form

44
Q

3 fold basis of therapy

A

Limit protein intake
Lactulose/levulose- prevent absorption of ammonia
Replace depleted/ missing enzyme

45
Q

DOC

46
Q

Benzoic acid binds with glycine

A

Phenylacetateglutamine

Biochem Prelim 2nd Sem (13 decks)

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