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19 Cards in this Set
- Front
- Back
What is beta oxidation, where does it occur?
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It is the oxidation pathway that burns FA
Very Long chain FA can only be burned in peroxisomes Long, medium, and short can be burned in Mito |
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How are fatty acids trapped inside cells, what enzymes are used to do this and where are these enzymes located?
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Fatty Acyl-CoA synthase is the enzyme that traps FA
Trapped by adding CoA to the FA Two steps – ATP added (as AMP) and then CoASH added (as CoA) |
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What compound is needed to move long-chain fatty acids into the mitochondrial matrix?
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Carnitine is used to move long chain FA into the mitochondria
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How is beta-oxidation in the mitochondria regulated?
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regulated by Malonyl CoA
Malonyl CoA inhibits Carnatine Palmitoyl Transferase I (CPTI) which causes the addition of Acetyl CoA to Carnitine |
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What is the most common genetic defect in fatty acid oxidation, what problems does it cause and how can it be detected at birth?
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Medium chain Acyl CoA dehydrogenase (MCAD)
Causes hypoketotic hypoglycemia Vomiting and lethargy after fasting in a child 3-15 months |
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What product is produced by the beta-oxidation of odd chain fatty acids and what vitamin is required for the conversion of this compound to glucose in the liver?
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Odd-chain FA produce Propionyl CoA
Requires B12 to convert it to glocose |
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What is alpha-oxidation?
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Occurs in peroxisomes
Handles odd carbon FA with methyl side chains (phytanic acid from chlorophyll) |
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what are the three end products that come from the alpha-oxidation of this compound?
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Acetyl CoA
Propionyl CoA Isobutyryl CoA |
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Compare beta-oxidation in mitochondria with beta-oxidation in peroxisomes:
Peroxisomes |
10% of all beta oxidation
Carnitine transport not needed Produce H2O2, NADH, and Acetyl CoA Handle very long chain FA No ATP production |
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Compare beta-oxidation in mitochondria with beta-oxidation in peroxisomes:
Mitochondria |
Produces ATP and NADH and CO2
Carnitine needed |
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What is omega-oxidation?
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Oxidation that starts at the farther most Carbon
Used to improve water solubility for the excretion in urine |
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where does Omega-Oxidation occur?
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Occurs in the ER using a mixed function oxidase
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what two vitamins and class of lipid are metabolized using omega-oxidation?
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Works on Eicosanoids, Vitamin E, and Vitamin A
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What are ketone bodies?
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Ketone bodies are formed from generation of large quantities of Acetyl-CoA in the liver
Ketone bodies can be formed from any aa and this can occur in any tissue |
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what conditions are needed for the production of Ketone Bodies?
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Starvation
Severe DM (??) Rapid mobilization of fat Need a high rate of beta oxidation in the liver to form them |
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Where and why are ketones used to produce energy?
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Used as energy by the heart, skeletal muscle, kidney and brain
Acetyl-CoA is used to make glucose for the brain |
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What conditions are needed for severe ketoacidosis to occur?
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An insulin deficiency and a glucagon excess cause accelerated ketogenesis
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what kind of increase in blood and urine levels of ketone bodies occur during severe ketosis?
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Excreted levels of ketones are 40 times higher in the urine and 30 times higher in the blood
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What 4 conditions can result in enough ketone body production to cause death?
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Uncontrolled diabetes mellitus (Type I without insulin – 3%)
Septic Shock (Type II) Myocardial Infarction (Type II) Pregnancy |