Lipid Metabolism

Question 1

Name lipid sources

Answer 1

diet, biosynthesis in liver, storage depots in adipocytes

Question 2

What is the structure of a fatty acid

Answer 2

hydrophilic carboxylic head and hydrophobic hydrocarbon tail

Question 3

What enzymes are involved in lipogenesis?

Answer 3

acetyl CoA carboxylase and fatty acid synthase

Question 4

What may be the basis for fatty acid oxidation disorders?

Answer 4

there are different families of acyl-CoA dehydrogenase that catalyse the initial beta-oxidation step depending on the carbon chain length e.g. short = less than 6C, medium = 6-12C, long = 13-21C and very long = above 21C

Question 5

Name some features of medium chain Acyl-CoA dehydrogenase deficiency

Answer 5

autosomal recessive, screened at birth, high carbohydrate diet, don’t fast

Question 6

Name some features of primary carnitine deficiency

Answer 6

autosomal recessive, SLC22A5 gene mutation, reduced ability to for cells to take up carnitine for beta-oxidation, muscle weakness