Flashcards in Beta Oxidation Deck (14):
SCFAs and MCFAs enter the mitochondria by:
LCFAs and VLCFAs enter the mitochondria by:
Enzymes of the carnitine shuttle (4)
1. Fatty Acyl CoA Synthetase (FACOAS)
2. CPT 1 (RLS)
4. CPT II
Fatty Acyl CoA Synthetase function
FA --> Fatty acyl CoA (moves inside the IMS)
CPT 1 function
AKA carnitine palmitoyltransferase I and carnitine acyltransferase.
Function is to add carnitine to fatty acyl CoA.
Allow fatty acyl carnitine to enter the mito matrix.
Remove carnitine and recreate fatty acid CoA
4 Steps of Beta Oxidation
Ox (ACAD), H (ECAD), O (3-HCAD), T (ACAAT or ketothiolase)
Beta oxidation of odd number FAs (4 steps)
1. Metabolize duntil propionayl-CoA remains (3 carbons)
2. Propionyl CoA carboxylase uses ATP to become methylmalonyl-CoA
3. Methylmalonyl mutase generate succinyl CoA
4. Succinyl CoA enters TCA
Beta oxidation of VLCFAs
Occurs in peroxisome and does not produce ATP.
Produced H2O2, but turned into O2 and H20.
First step is catalyzed by acyl CoA oxidase.
Impairs breakdown of MCFAs.
Leads to secondary carnitine deficiency, so FAs accumulate in liver.
Patients must depend on Glc as source of energy.
3 Ketone bodies
Produced in liver only.
Mild to moderate increase of ketones bodies. Occurs in mild fasting, during pregnancy and in babies.