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19 Cards in this Set

  • Front
  • Back
What is beta oxidation, where does it occur?
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
How are fatty acids trapped inside cells, what enzymes are used to do this and where are these enzymes located?
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)
What compound is needed to move long-chain fatty acids into the mitochondrial matrix?
Carnitine is used to move long chain FA into the mitochondria
How is beta-oxidation in the mitochondria regulated?
regulated by Malonyl CoA
Malonyl CoA inhibits Carnatine Palmitoyl Transferase I (CPTI) which causes the addition of Acetyl CoA to Carnitine
What is the most common genetic defect in fatty acid oxidation, what problems does it cause and how can it be detected at birth?
Medium chain Acyl CoA dehydrogenase (MCAD)
Causes hypoketotic hypoglycemia
Vomiting and lethargy after fasting in a child 3-15 months
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?
Odd-chain FA produce Propionyl CoA
Requires B12 to convert it to glocose
What is alpha-oxidation?
Occurs in peroxisomes
Handles odd carbon FA with methyl side chains (phytanic acid from chlorophyll)
what are the three end products that come from the alpha-oxidation of this compound?
Acetyl CoA
Propionyl CoA
Isobutyryl CoA
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
Compare beta-oxidation in mitochondria with beta-oxidation in peroxisomes:
Mitochondria
Produces ATP and NADH and CO2
Carnitine needed
What is omega-oxidation?
Oxidation that starts at the farther most Carbon
Used to improve water solubility for the excretion in urine
where does Omega-Oxidation occur?
Occurs in the ER using a mixed function oxidase
what two vitamins and class of lipid are metabolized using omega-oxidation?
Works on Eicosanoids, Vitamin E, and Vitamin A
What are ketone bodies?
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
what conditions are needed for the production of Ketone Bodies?
Starvation
Severe DM (??)
Rapid mobilization of fat
Need a high rate of beta oxidation in the liver to form them
Where and why are ketones used to produce energy?
Used as energy by the heart, skeletal muscle, kidney and brain
Acetyl-CoA is used to make glucose for the brain
What conditions are needed for severe ketoacidosis to occur?
An insulin deficiency and a glucagon excess cause accelerated ketogenesis
what kind of increase in blood and urine levels of ketone bodies occur during severe ketosis?
Excreted levels of ketones are 40 times higher in the urine and 30 times higher in the blood
What 4 conditions can result in enough ketone body production to cause death?
Uncontrolled diabetes mellitus (Type I without insulin – 3%)
Septic Shock (Type II)
Myocardial Infarction (Type II)
Pregnancy