Lipids I

Question 1

Fatty acids

Answer 1

alkyl chain with terminal carboxyl group, even number of carbons in humans

Question 2

Triglycerides

Answer 2

3 FAs esterified to a glycerol

Question 3

trans FA in diet

Answer 3

cannot be metabolized well in humans, results from hydrogenation. Has neg. efects like cardiovascular disease.

Question 4

Naturally occurring FAs

Answer 4

have double bonds in cis formation.

Question 5

Essential FAs

Answer 5

double bonds passt carbon P. Examples are linolenic acid (omega 3) and linoleic acid (omega-6)

Question 6

Coenzyme A

Answer 6

FA is activated by binding to coenzyme A in ATP-dependent reaction by enzyme Acyl-CoA synthetase

Question 7

Carninte

Answer 7

carrier to bring GA across membrane. Takes acyle away from CoA

Question 8

Degradation of branched FA

Answer 8

use peroxisomal alpha-oxiation in peroxisomes

Question 9

Carnitine deficieny

Answer 9

cannot transfer FA into mitochondria. Results in hypoglycemia and low ketone levels in urine

Question 10

MCAD defiency

Answer 10

acyl-CoA dehydrogenase deficiency. Presents as non-ketotic hypoglycemia and can be life threating

Question 11

Refsum disease

Answer 11

problem in alpha-oxidation of branched FA phytanic acid. Causes accumulation of undegraded phytanic acid in blood and tissues, causes neurologica defects

Question 12

Enzymatic defect leading to development of acute fatty liver of pregnancy

Answer 12

defefct in LCAD. Stops degradation of long FA = accumulates in cytoplasm of mother’s liver