Exam 30: Amino Acid Degradation and the Urea Cycle

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Which of the following is an allosteric activator of mammalian carbamoyl phosphate synthetase?

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What type of damage occurs if there is a defect in the urea cycle?

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A defect in this cycle causes hyperammonemia (large amount of NH4+). Genetic defects become obvious immediately. After lethargy and vomiting begins, coma and brain damage follow, most likely caused by the high levels of glutamate and glutamine in the brain.

Uricotelic organisms release nitrogen as:

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Choose the correct answer from the list below. Not all of the answers will be used. -The product _______________ results when aspartate is transaminated with ɑ-ketoglutarate.

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Choose the correct answer from the list below. Not all of the answers will be used. -The ______________ enzymes transfer an ɑ-amino group from amino acids to ɑ-ketoglutarate.

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The genetic deficiency of the enzyme ___________________ results in a condition referred to as phenylketonuria.

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In the degradation of amino acids, the amino nitrogens can eventually become the amino group of ___________________.

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Choose the correct answer from the list below. Not all of the answers will be used. -Serine and threonine are deaminated by the enzyme _______________.

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Write the net equation of the urea cycle and indicate whether energy is produced or consumed by the cycle.

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What is the basic strategy by which proteins are degraded?

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Why do you think that weight lifters take large amounts of branched-chain amino acids?

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Which amino acids can be converted to pyruvate by a single enzyme-catalyzed reaction?

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The hydrolysis of arginine by arginase produces ornithine and ___________________.

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Choose the correct answer from the list below. Not all of the answers will be used. -The methyl group donor _______________ is the product of the first step of methionine degradation.

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In the first step of the urea cycle, CO2 and NH4+ are converted into ___________________.

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In the urea cycle, the second nitrogen of urea enters the cycle in the form of which of the following metabolites?

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In addition to increased fatty acid accumulation in the liver, excess consumption of ethanol can also induce two further stages of liver damage. What are they?

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Which amino acids can be directly deaminated to produce NH4+?

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What is the cause of PKU? Give the structure of the abnormal metabolite that accumulates from which this condition gets its name.

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Which amino acids supply carbons for eventual entry into metabolism as succinyl CoA?

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