Beta Oxidation Flashcards
What is the end-goal of Fatty Acid Oxidation?
Break Fatty Acids down into Acetyl CoA to form Ketones & other Metabolic Fuel.
The size of the Fatty Acid you are breaking down determines what?
Where it is oxidized.
What are the 3 different Fatty Acid Sizes?
How many carbons do Short/Medium Chain Fatty Acids Have?
2-12 Carbons
How many carbons do Long Chain Fatty Acids Have?
14-20 Carbons
How many carbons do Very Long Chain Fatty Acids Have?
> 20 Carbons
Where/how does the location of Beta-Oxidation of short/medium chain fatty acids occur?
Diffuse Freely into Mitochondria
Where/how does the location of Beta-Oxidation of long chain fatty acids occur?
Must use Carnitine Transport into Mitochondria.
Where/how does the location of Beta-Oxidation of very long chain fatty acids occur?
Must be oxidized in a peroxisome
Carnitine Transport can be thought of as what with regard to Beta Oxidation?
Carnitine Transport is the precursor to beta-oxidation. (precedes it)
Before Beta Oxidation occurs, where does the Fatty Acid originate from?
Cytoplasm
What is the 1st step of Carnitine Transport?
Fatty Acid is converted into Fatty Acyl-CoA
What enzyme catalyzes a Fatty Acid into Fatty Acyle-CoA?
Fatty Acyl CoA Synthetase
How does a Fatty Acid make it’s way into the mitochondrial membrane?
It can’t cross until it is converted into Fatty Acyle-CoA which is permeable to the mitochondrial membrane (diffusion).
How does a Fatty Acid make it’s way into the mitochondrial membrane?
It can’t cross until it is converted into Fatty Acyle-CoA which is permeable to the mitochondrial membrane (diffusion).
What is the 2nd step of Carnitine Transport?
Fatty Acyl-CoA is converted into Fatty Acyl-Carnitine
What enzyme catalyzes a Fatty Acyl-CoA into Fatty Acyl-Carnitine?
Carnitine Acyltransferase-1 (“CAT-1”)
Once the Carnitine is attached to the Fatty Acyl, what is then allowed to happen?
It is allowed to enter the mitochondria.
What inhibits the Fatty Acyl-Carnitine from being catalyzed and why?
Presence of Malonyl CoA in mitochondria will inhibit b/c Malonyl CoA is a product of Fatty Acid Synthesis which will inhibit fatty acid breakdown.
After Fatty Acyl-Carnitine enters the mitochondria, what is the 3rd step of Carnitine Transport?
Fatty Acyl-Carnitine is converted into Fatty-Acyl-CoA + Carnitine
What enzyme catalyzes Fatty Acyl-Carnitine into Fatty Acyl-CoA + Carnitine?
Carnitine Acyltransferase-2 (“CAT-2”)
The Carnitine Shuttle System of Beta Oxidation is basically converting Fatty Acid into what?
Fatty Acyl-CoA & moving it into the mitochondria.
What is the first step of Beta Oxidation?
Fatty Acyl-CoA is converted into Acetyl CoA
What enzyme converts Fatty Acyl-CoA into Acetyl CoA?
Fatty Acyl CoA Dehydrogenase
Once you have Acetyl CoA, what two products can it be converted into?
Ketone Bodies for the Liver, or used to spin the TCA cycle to generate ATP & ETC substrates.
What is the Rate Limiting Enzyme of Beta Oxidation?
CAT-1 (during Carnitine Transport)
With Systemic Primary Carnitine Deficiency, what can you expect to see?
- No Transport into Mitochondrial Membrane
- Hypoketotic Hypoglycemia
With Myopathic CAT-2 Deficiency, what can you expect to see?
No CAT-2
- Myoglobinuria
- Hypotonia, weak
- Increase Trigylcerides in muscles
