LECTURE 29 - UREA CYCLE Flashcards

1
Q

which groups serve as co factors for transaminases?

A

PLP
PMP
Vit B6

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2
Q

which enzymes prefer alpha KG as a substrate?

A

glutamate dehydrogenase
aspartate transaminase
tyrosine aminotransferase

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3
Q

who discovered the urea cycle

A

first metabolic pathway defined
discovered by hans adolf krebs and hanseleit
hans krebs did the CAC

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4
Q

what are the sources of NH4+ in the urea cycle?

A

comes from glutamine (glutaminase) and glutamate (glutamate dehydrogenase)

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5
Q

in which cell compartments does urea cycle happen?

A

mitochondria and cytoplasm

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6
Q

what is the rate limiting of the urea cycle/

A

carbamoyl phosphate synthetase 1 (CPS1)
makes carbamoyl phosphate
CPS1 uses 2ATP and HCO3- which comes from CAC (which is also in the mitochondria!)
carbamoyl phosphate is the first substrate of the urea cycle
happens when high ATP concentration

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7
Q

what is the CPS1 reaction?

A

creates a high energy intermediate

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8
Q

how is CPS1 regulated?

A

allosterically activated by N-acetylglutamate (NAG)
NAG is produced by NAG synthase (NAGS)
NAGS is stimulated by arginine
NAG production is proportional to concentration of arginine
more arginine = more urea cycle, activates CPS1

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9
Q

synthesis of NAG

A
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9
Q
A
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10
Q

CPS2 vs CPS1

A
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11
Q

what are the different ways humans can have hyperammonia

A
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12
Q

what is the first step of the urea cycle?

A

ornithine and citrulline use the same transporter

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13
Q

what is step 2 of the urea cycle?

A
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14
Q

what are steps 3 and 4 of the urea cycle?

A
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15
Q

where do the nitrogens come from?

A

aspartate transaminase, which makes aspartate from oxaloacetate is found in mitochondria of liver
the oxaloacetate comes from the CAC, and the fumarate will join back into the CAC

16
Q

graphic that explains link between urea cycle and CAC

A
17
Q

what is the energy cost and energy gain of the urea cycle?

A
18
Q

what are the by products of the urea cycle?

A

urea
fumarate
alpha KG