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Block 6- Urea cycle

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Question
Answer
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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