Beta oxidation

Question 1

What is the first step?

Answer 1

Acyl CoA produced from fatty acid plus ATP plus HS-CoA. Catalyzed by acyl CoA synthetase

Question 2

Where does Acyl CoA generation take place?

Answer 2

Outer mitochondrial membrane

Question 3

What molecule is produced when carnitine is coupled to acyl CoA?

Answer 3

Acyl carnitine

Question 4

What is the transport protein called?

Answer 4

Translocase

Question 5

Primary carnitine deficiency?

Answer 5

Autosomal recessive disorder. Symptoms appear early and include muscle weakness, cardiomyopathies and hypoglycemia. Gene SLC22A5 is mutated and carnitine transporter has a reduced ability

Question 6

4 reactions in the oxidation cycle?

Answer 6

Oxidation, hydrolysis, oxidation and thiolysis

Question 7

Net gain of ATP from one palmitate molecule?

Answer 7

129 (35 ATP for Beta oxidation, 96 ATP for 8 acetyl CoA undergoing TCA cycle and minus 2 for generation of acyl CoA)

Question 8

How many acetyl CoA molecules are produced from one palmitoyl CoA molecule?

Answer 8

8

Question 9

When fat breakdown predominates, what does acetyl CoA form?

Answer 9

acetoacetate, D-3-hydroxybutyrate and acetone (known as ketone bodies)