Carnitine Shuttle W1 Flashcards
What is the carnitine shuttle?
Aids in transportation of long-chain fatty acids into mitochondria for beta oxidation
Where is carnitine derived from?
Biosynthesized from lysine and methionine
Why is the carnitine shuttle required?
Due to the mitochondrial membrane being impermeable to long-chain FAs
What does Acyl-CoA synthetase?
It utilises cofactors ATP and CoASH
It adds a coenzyme A group to the long-chain fatty acid and activates the FA
This is then activated via CPT1
What does CPT1 do?
It removes CoASH from fatty cell chain
It adds carnitine to fatty cell chain
When CPT 1 adds a carnitine to the fatty cell chain, what does this allow the chain to do?
Enter the intermembrane space
What part of the fatty SL chain allows it to cross the intermembrane space to the mitochondrial matrix?
The carnitine part of the fatty acyl carnitine
What does Carnitine-Acylcarnitine Translocase do?
Allows the fatty acyl carnitine to cross the inner mitochondrial membrane into the mitochondrial matrix
It attaches on enzyme CPT2
What does CPT2 do?
It takes CoASH and removes carnitine from fatty acyl
Adds Coenzyme A to fatty acyl chain
Helps regenerate carnitine
Opposite direction to CPT1
When does beta oxidation occur?
When we have fatty CoA in the mitochondrial matrix
What enzyme acts on carnitine?
Acetyltransferase
What does beta oxidation produce?
Acyl as a product
What does Acetyltransferase do?
Transfers an acetyl group to carnitine to gives acetyl carnitine
Where can the acetyl group derive from to create acetyl carnitine?
Acetyl CoA generated via beta oxidation of the fatty acyl chain