Mitochondrial Targeting

Question 1

What is step 1 of mitochondrial targeting?

Answer 1

1) proteins kept unfolded by chaperones. (Mitochondral Import Stabilisation Factor in cytosol)

Question 2

What is step 2 of mitochondrial targeting?

Answer 2

they contain amphiphatic N terminal signal which is recognised by TOM to form a protein import channel

Question 3

What is step 3 of mitochondrial targeting?

Answer 3

Proteins destined for matrix transported across inner membrane by TIM, (ATP dependant process). Proteins destined to stay in membrane have additional signals to stop transport.

Question 4

What is step 4 of mitochondrial targeting?

Answer 4

N-terminal cleaved by Mitochondrial Processing Peptidase so a protein folds to native conformation (chaperones such as Hsp70 aid this process in an ATP dependent manner).

Question 5

What is pyruvate dehydrogenase deficiency?

Answer 5

build up of lactic acid causing neurological problems