Exam 30: Amino Acid Degradation and the Urea Cycle
How is the urea cycle linked to the citric acid cycle?
During the urea cycle, aspartate brings in the second nitrogen by combining with citrulline to form argininosuccinate. Then argininosuccinate is cleaved, forming arginine and fumarate. The fumarate is then converted into malate and then oxidized to oxaloacetate, which can be transaminated to aspartate or serve as a precursor to glucose. Fumarate, malate, and oxaloacetate are all intermediates of the citric acid cycle. Figure 30.3 in the text shows this integration.
Describe the glucose-alanine cycle and its significance in amino acid metabolism.
During prolonged exercise and fasting, muscles use branched-chain amino acids as fuel. This degradation takes place in the muscle, but conversion to urea is not possible in this tissue, thus transport to the liver is required. The amino nitrogen is removed by transamination to produce glutamate. The glutamate then transaminates with pyruvate, producing alanine, which is released into the blood and transported to the liver. In the liver, the alanine is converted to pyruvate and the urea is metabolized to urea. The pyruvate is converted to glucose by gluconeogenesis and the glucose is transported back to the muscle via the bloodstream.
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-The molecule _______________ is oxidatively deaminated to an ammonium ion.
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Which amino acids can be converted to pyruvate by a single enzyme-catalyzed reaction?
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-The molecule _______________ is formed from excess NH4+ by ureotelic organisms.
Which amino acid(s) is/are converted to tyrosine in the degradative pathway?
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-The methyl group donor _______________ is the product of the first step of methionine degradation.
Write the net equation of the urea cycle and indicate whether energy is produced or consumed by the cycle.
The genetic deficiency of the enzyme ___________________ results in a condition referred to as phenylketonuria.
Why are more uricotelic organisms found in the desert than in the tropics?
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-Serine and threonine are deaminated by the enzyme _______________.
Which of the following is an allosteric activator of mammalian carbamoyl phosphate synthetase?
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-The _______________ enzyme catalyzes an oxidative deamination and can utilize either NAD+ or NADP+.
In the urea cycle, the second nitrogen of urea enters the cycle in the form of which of the following metabolites?
What is the cause of PKU? Give the structure of the abnormal metabolite that accumulates from which this condition gets its name.
Oxidative deamination of glutamate results in free ammonium ions and ___________________.
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