the urea cycle Flashcards

1
Q

true/false: all pathways for amino acid degradation include the separation of the alpha-amino group

A

true

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2
Q

what are the two themes of amino acid catabolism

A

N theme - catabolism of amino groups
C theme - catabolism of carbon skeletons

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3
Q

how are amino acids broken down in order to enter the citric acid cycle?

A

amino acids are broken down to acetyl-coA which then gets into the citric acid by getting converted to citrate, or doesn’t get to the citric acid cycle and becomes ketone bodies

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4
Q

when amino acids are used to replenish intermediates in the citric acid cycle, this is referred to as:
a) cataplerosis
b) anaplerosis
c) aminoplerosis
d) ketosis

A

b

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5
Q

this enzyme catalyzes the combination of free ammonia with glutamate to yield glutamine; requires ATP and it is critical in order to transport toxic ammonia to the liver

A

glutamine synthetase

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6
Q

this enzyme catalyzes the conversion of glutamine to glutamate and NH4+

A

glutaminase

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7
Q

_____ muscles produce pyruvate, lactate and ammonia

A

skeletal

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8
Q

this enzyme interconverts pyruvate and alanine via transamination with glutamate

A

alanine aminitransferase

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9
Q

in the _________ cycle, alanine carries ammonia and the carbon skeleton from pyruvate to the liver

A

glucose-alanine

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10
Q

in the glucose-alanine cycle, _______ is excreted

A

ammonia

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11
Q

in the glucose-alanine cycle, _______ is used to produce glucose which is returned to the muscle

A

pyruvate

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12
Q

involves muscle protein being degraded to provide more glucose to generate additional ATP for muscle contraction.

A

the glucose alanine cycle

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13
Q

amino acids are not stored in appreciable concentrations. however, which amino acid would be found in significant concentration in the blood of basketball players after a long and intense workout in a gym?
a) histidine
b) alanine
c) glutamine
d) cysteine

A

b

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14
Q

what is the primary role of the glucose-alanine cycle?
a) converting glucose to alanine
b) delivering amino groups from the muscle to the liver
c) delivering de novo synthesized glucose from the liver to the muslce
d) enhancing protein degradtion
e) preventing glucose accumulation in the muscle

A

b

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15
Q

urea is produced from ammonia in 5 enzymatic steps: this enzyme brings in ammonia from deamination reactions

A

step 1: carbamoyl phosphate synthetase I

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16
Q

urea is produced from ammonia in 5 enzymatic steps: this enzyme brings carbamoyl phosphate into the urea cycle. resulting citrulline can enter cytosol

A

step 2: ornithine transcarbamoylase

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17
Q

urea is produced from ammonia in 5 enzymatic steps: this enzyme brings in second amino group from aspartate

A

step 3: argininosuccinate synthetase

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18
Q

urea is produced from ammonia in 5 enzymatic steps: this enzyme removes length of carbon skeleton as fumerate

A

step 4: argininosuccinase

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19
Q

urea is produced from ammonia in 5 enzymatic steps: this enzyme cleaves arginine to form urea and ornithine (back at the beginning of the cycle)

A

step 5: arginase

20
Q

urea is produced from ammonia in 5 enzymatic steps: this is the rate limiting step. the reaction has two activation steps that require ATP. the second step of the reaction uses ammonia from deamination reaction

A

the carbamoyl phosphate synthetase I reaction

21
Q

what is the first step of the carbamoyl phosphate swynthetase I reaction?

A

bicarbonate is phosphorylated by ATP (activation step)

22
Q

what is the second step of the carbamoyl phosphate synthetase I reaction

A

ammonia from deamination reactions displaces it phosphoryl group from bicarbonate to generate carbamate

23
Q

what is the third step of the carbamoyl phosphate synthestase I reaction

A

`carbamate is phosphorylated from ATP to yield carbamoyl phosphate

24
Q

______ catalyzes the formation of citrulline and Pi from ornithine and carbamoyl phosphate

A

ornithine transcarbamoylase

25
Q

the urea cycle:
a) can be used to make uric acid by oxidation of urea in the liver
b) occurs primarily in the kidneys
c) can be used ti generate NH4+ from urea
d) uses two unusual alpha-amino acids as intermediates

A

d

26
Q

where does the urea cycle occur?

A

in the mitochondria of the liver cells

27
Q

what is the main function of the urea cycle

A

to remove toxic ammonia from the body

28
Q

true/fasle: the formation of argininosuccinate requires atp

A

true

29
Q

______ catalyzes the condensation of the amino group from aspartate and the ureido group from citrulline to form argininosuccinate

A

argininosuccinate synthetase

30
Q

what is the intermediate of the formation of argininosuccinate

A

citrullyl-AMP intermediate

31
Q

true/false: in order to form argininosuccinate, citrulline needs to pass from the mitochondria and go into the cytosol

A

true

32
Q

________ catalyzes the reversible cleavage of argininosuccinate to form arginine and fumerate

A

argininosuccinase

33
Q

what happens to fumerate once it formed from argininosuccinate

A

fumerate gets converted to malate and joins the pools of other citric acid cycle intermediates

34
Q

is the formation of arginine reversible or irreversible

A

reversible

35
Q

______ catalyzes the cleavage of arginine to form urea and ornithine.

A

arginase

36
Q

where does the formation of urea from arginine occur

A

cytosol

37
Q

where does the formation of arginine from argininsuccinate occur

A

cytosol

38
Q

what happens to ornithine once it is formed from arginine

A

ornithine gets transported into the mitochondria to initiate another round

39
Q

two nitrogen containing groups are used to make urea in liver cells. which two molecules of the urea cycle contribute to these two groups?
a) carbamoyl phosphate and aspartate
b) alanine and glutamine
c) glutamate and glutamine
d) carbamoyl phosphate and glutamate
e) arginine and aspartate

A

a

40
Q

what links the citric acid cycle and the urea cycles together

A

aspartate-argininosuccinate shunt - links the amino groups and the carbon skeletons together

41
Q

which molecule that is produced in the cytosol by the urea cycle can be used by the citric acid cycle but cannot pass the inner mitochondrial membrane?
a) malate
b) argininosuccinate
c) ornithine
d) NAD+
e) fumerate

A

e

42
Q

does N-acetylglutamate inhibit or stimulate carbamoyl phosphate synthetase I

A

stimulates

43
Q

N-acetylglutamate synthase catalyzes the formation of N-acetylglutamate from ______ and _____

A

acetyl-coa and glutamate

44
Q

how is the urea cycle regulated
a) a diet rich in carbohydrates and lipoids results in downregualtion of urea cycle enzymes
b) arginine stimulate the urea cycle by activated N-acetylglutamate synthase
c) starvation or ingestion of protein-rich diets up regulates urea cycle enzymes
d) carbamoyl phosphate synthetase I is allosterically regulated by N-acetylglutamate
e) all of the above

A

e

45
Q

what are the two main forms of regulation of the urea cycle

A
  1. at the level of enzyme synthesis for the urea cycle enzymes and carbamoyl phosphate synthetase I
  2. by allosteric regulation of carbamoyl phosphate synthetase I