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24 Cards in this Set

  • Front
  • Back
What does Beta oxidation of FA do?
Catabolism of FA to form Acetyl CoA-->TCA or ETC
Where does beta oxidation of FA occur?
In mitochondrial matrix
What are the steps of Beta Oxidation?
1. Activation
2. Carnitine cycle
3. Dehydrogenation
4. Hydration
5. Dehydrogenation
6. Cleavage
Sources of free FA
1. Dietary fat
2. Stored fat (TAG)-released by lipase
3. Phospholipids
Activation of free FA occurs where?
-Occurs in the outer mitochondrial membrane
Fatty acid + ATP-->Fatty acid-CoA
Fatty acyl-CoA Synthetase
cleavage of PPi-->2Pi makes rxn irreversible
Carnitine acyl transferase I (CAT I, regulated)
-Found on outer mitochondrial membrane
-Fatty-acyl CoA + carnitine --> CoA + fatty acid-carnitine
Carnitine acyl transferase II (Cat II, unregulated)
-Found on inner mitochondiral membrane
-Fatty acid-carnitine + CoA --> carnitine + fatty acyl CoA
1. Acyl-CoA --> Enoyl-CoA
alkane --> alkene
Acyl-CoA dehydrogenase
Dehydrogenation (oxidation)
2. Enoyl-CoA + H2O -->B-hydoxyacyl CoA
Double bond --> Alcohol
Enoyl CoA hydratase
3. B-hydroxyacyl-CoA --> B-ketoacyl-CoA
Alcohol --> ketone
B-hydroxyacyl-CoA dehydrogenase
4. B-ketoacyle CoA --> acetyl CoA
B-keto thiolase
Enoly CoA isomerase
-Unsaturation in wrong place and/or wrong configuration
-for odd number
Reductase and enoyl CoA isomease
-Removes an additional double bond and transfers another to the right place
-even numbered
Odd number of carbons in acyl chain
6 acetyl CoA + 1 propionyl CoA (3C)
Regulation of B-oxidation when starvation
free FA levels increases bc of lipases which are stimulated by epinephrine and glucagon
Regulation of B-oxidation when well fed
FA decrease due to inhibition of adipose cell lipase by insulin
Regulation of CAT I
Inhibited by high concentrations of malonyl CoA
Inhibition of B-hydroxyacyl CoA dehydrogenase by?
NADH (its product)
Inhibition of B-ketothiolase by?
Acetyl CoA (its product)
Carnitine deficiencies
Decreased ability of tissues to utilize long-chain fatty acids

Accumulation of toxic amounts of FA
Medium-chain fatty acyl CoA dehydrogenase (MCAD) deficiency
-4 specific fatty acyl CoA dehydrogenases
-Common inborn error of metabolism
-Decrease FA oxidation-->hypoglycemia
-Treatment: carbohydrate-rich diet
Excess oxidation
When fat is the main energy source (starvation, diabetic)

Produce ketone bodies
Cis vs. trans FA
-Cis double bonds increase membrane fluidity and trans double bonds pack well and decrease fluidity.

Accumulate in the membrane