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How is aspartate made? (from what 2 compounds and which enzyme)
Glutamate and OAA catalyzed by AST
What would a ornithine trascarbamoylase (OTC) deficiency lead to?
How does phenylbutyrate treat hereditary hyerammonemia?
It is converted to phenylacetate in the liver and the liver conjugates phenylacetate with glutamine and excretes it in the urine so ammonia (part of pheylacetylglutamine) is secreted with it
In a patient with OTC deficiency, what would daily exogenous citrulline do?
It would allow the urea cycle to continue getting rid of one ammonia from aspartate and one ammonia would be from the exogenous citrulline (it would help decrease hyperammonemia)
What is carnitine used for in fatty acid metabolism?
Carnitine shuttle, it moves long-chain fatty acids from the cytosol to the mitochondria matrix for beta-oxidation
How will a carnitine deficiency affect lipolysis?
Carnitine deficiency leads to hypolgycemia which leads to low insulin levels which stimulates lipolysis
What will a carnitine defeciency do to beta-oxidation?
No affect because it is a carnitine deficiency causing hypoglycemia
What type of hyperlipidemia would you suspect in a patient with a thick creamy top layer on a blood specimen after centrifugation?
hyperchylomicronemia, chylomicrons are less dense and will float
Normally, where is LPL found?
Attached to the glycocalyx on endothelial cells
What does lipoprotein lipase deficiency lead to?
elevated levels of CMs and TGs, hyperchylomicronemia (type I hyperlipidemia)
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