A newborn is brought into a clinic, and his parents indicate that their baby is lethargic, vomits periodically, and has tremors. It is suspected that the baby might have hyperammonemia. The cause may be due to urea cycle defects, indicating that the high ammonia level is due to an enzymatic block in ammonia metabolism within the urea cycle. What are the enzymes of the urea cycle? Describe their reactions in order to understand the cause of this phenomenon?
Q: Illustrate the urea cycle. Briefly explain the process.
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Q: Which of the following is common shared compound by the TCA and Urea cycle?
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- Discuss the toxic effects of the ammonium ion and excess bicarbonate ion. What is the effect of high concentrations of NH4+ in the bloodstream? What are the two reasons NH4+ is so toxic to the brain? Why can the urea cycle also be considered as a bicarbonate ion disposal mechanism? Why is that important?Which of the following statements regarding the urea cycle is true? (A) Fumarate acts as a connecting link between urea cycle and the CAC.(B) The urea cycle occurs solely in the mitochondria.(C) Citrulline and arginine reacts to form argininosuccinate.(D) Ornithine formed in the last reaction of the urea cycle is excreted from the body along with urea.(E) The purpose of the urea cycle is to produce energy (4 ATP per ammonia that enters) for the liver.Which of the following is a substrate in the urea cycle? a. fumarate b. glutamate c. aspartate d. α-ketoglutarate
- Production of the enzymes that catalyze the reactions of the urea cycle can increase or decrease according to the metabolic needs of the organism. High levels of these enzymes are associated with high-protein diets as well as starvation. Explain this apparent paradox.Consider the following Statements: (1) Citric acid cycle intermediates are often the products from amino acid carbon skeleton degradation. (2) The net effect of transamination is to collect the amino groups from a variety of amino acids into the amino acids glutamate and aspartate. (3) All of the reactions of the urea cycle occur within mitochondria. how many is trueMammals excrete most nitrogen atoms as urea. The urea cycle itself requires considerable amounts of ATP. What mechanism does the liver cell have to compensate for that energy input?
- hyperammonemia in patients is compounded by the reality of the normal regulatory controls in the urea cycle. Explain how increased GDH oxidative deamination activity will inhibit the urea cycle.What enzyme catalyzes the committed reaction of the urea cycle? carbamoyl phosphate synthetase II carbamoyl phosphate dehydrogenase I carbamoyl phosphate synthetase I carboxyphosphate synthetase II carboxyphosphate synthetase IWhich amino acids in the urea cycle are the links to thecitric acid cycle? Show how these links occur