Please explain your reasoning for each answer! Thank you. A significant number of children are born with muscle weakness and necrosis, hypoglycemia, elevated ammonia in the blood (hyperammonemia), fatigue and heart disorders. These children also may have elevated acyl-carnitine in their blood, muscle and liver. As a result, all newborns are tested for the condition that is the focus of this project. (Tip: think about glycolysis: what pathways are regulated together? What pathways are increased in rate together when energy (or other significant molecules) are low or high in concentration?) (a) How can carnitine and acyl-carnitine levels be accurately determined in blood, urine or in a tissue biopsy? Give at least one laboratory method that is actually used for each molecule (b) The central theme of this medical condition is on the role of carnitine. Why is acyl-carnitine increased in concentration when free carnitine is low in concentration in all parts of liver cells (although there is one location on which you should really concentrate!)? (c) Based on your answer to part (b), please explain the presentation in the clinic. That is, what led to low glucose, high fatty acids (i.e. acyl-carnitine) in liver cell cytosol and elevated levels of ammonia when you consider the metabolic pathways that you know and specifically the β-oxidation (fatty acid oxidation), gluconeogenesis, and the urea cycle?


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