What can cause the accumulation of lipids in the liver of patients with SCD?

Free fatty acids travel to the liver to undergo gluconeogenis yielding glucose and acetyl CoA
through beta- oxidation. The Kreb’s cycle produces ATP and CO2 and ketogenesis produces
ketone bodies. Individuals suffering from SCD accumulate fatty acids in the liver because they
can not oxidize long-chain fatty acids through gluconeogenesis.

ree fatty acids travel to the liver

to undergo gluconeogenis
yielding glucose and acetyl
CoA through beta- oxidation.
The Kreb’s cycle produces ATP
and CO2 and ketogenesis
produces
ketone bodies. Individuals
suffering from SCD accumulate
fatty acids in the liver because
they
can not oxidize long-chain fatty
acids through gluconeogenesis.
Question 6 A decreased amount of carnitine in the liver and skeletal muscle causes inability to
produce ketone bodies and an increased amount of liver and muscle lipids. The urea cycle is
impaired within the liver because of an inability to oxidize long-chain fatty acids, which causes
hypoglycemia.

Question 7

During periods of fasting, carbohydrates are depleted an alternate source of glucose is needed for
energy metabloism. The liver is the site of most glucogenic activity. Gluconeogenesis creates
glucose from the catabolism of non-carbohydrates. One non-carbohydrate source is lipids.
Long-chained fatty acids lipids must undergo beta-oxidation in order to the create acetyl-coa and
ketone bodies used in energy metabolism. The long chain fatty-acids must be transferred into the
mitochondrial matrix from the cytosol in order to undergo beta-oxidation.

Carnitine is responsible for transferring long-chain fatty acids from the cytosol across the inner
mitochondrial membrane into the mitochondria matrix. In systemic carnitine deficiency there is
a defect in carnitine biosynthesis therefore LCFA cannot be transferred into the mitochondrial
matrix. Since the LCFA cannot be transferred into the mitochondrial matrix, beta-oxidation
cannot occur and this leads an increase accumulation of lipids in the liver because acetyl-coa
cannot be produced. Ketone bodies are derived mostly from the acetyl-coa formed in the liver
but since there is no beta-oxidation there is also a lack ketone bodies