Block 6- Urea cycle
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Where is urea synthesized? | liver
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What is the structure of urea? | 2 amino groups attached to a central carbonyl group
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Where do the parts of urea come from? | Carbonyl derived from CO2, 1 N-atom from aspartate (asp) the other from ammonium
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Where does Urea synthesis begin and end within the cell? | Mitochondria to start, cytoplasm to finish
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How much energy is required to run the urea cycle? | 4 ATPs
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What is the crucial, rate limiting first enzyme for urea synthesis (found in mitochondria)? | carbamoyl phosphate
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What is the second enzyme of the urea cycle? Also rate limiting | Ornithine transcarbamoylase condenses carbamoyl phosphate with ornithine to form citruline
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The most common genetic defiencies in urea cycle are associated with what? | the two enzymes: carbamoyl phosphate & ornithine transcarbamoylase
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What does arginase do in the urea cycle? Where? | cleaves arginine into urea and ornithine (which is recycled.
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Where does apspartate occur in the urea cycle? | It enters in the cytoplasm to convert citrulline to argininosuccinate which cleaves to form fumarate and arginine by a lyase.
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Is all the arginine required available via urea synthesis? | No, most comes from diet.
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What happens with CPS-1 and Ornithine transcarbamoylase deficiency? | Hyperammonenemia; Decrease in BUN (Blood Urea Nitrogen, Cerebral edema;lethargy, convulsion, coma, death
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What is the difference between Aspartate and Fumarate? | fumarate does not have the amino group
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Where does fumarate go following the urea cycle? | It can enter the TCA for energy or gluconeogenesis
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Where does urea go from the kidney? | Transported by blood to the kidney
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What is the famous phrase regarding the urea cycle? | Better to be pissed off, than pissed on
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Created by:
El Diablo
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