Lipid Metabolism

Question 1

MCAD Deficiency

Answer 1

impairs breakdown of MCFAs
secondary carnitine deficiency (low levels of carintine, due to excessive excretion of MCA carnitines in urine- the fatty acids bind to the carinitine and take it out of the body with them!)
C8 FA accumulates in liver, poisonous, interferes with urea cycle, elevated levels of ammonia
Patients depend on glucose as energy source

Treatment mainly preventative
Avoid fasting and other situations where the body relies on FA-b-oxidation to supply energy

Question 2

What are the Water-Soluble and Acidic Ketone Bodies

Answer 2

Acetoacetate
b-Hydroxybutyrate
Acetone

Question 3

Where are water soluble and acidic ketone bodies produced?

Answer 3

Produced in Liver Only

Question 4

Where are ketone bodies used?

Answer 4

Provide energy for peripheral tissues and during fasting & brain!!!! during starvation