Complex Lipid Metabolism

Question 1

Triacylglycerol

Answer 1

3 carbon-acyl groups with 3 fatty acid groups (one per a carbon)

Question 2

Phospholipids

Answer 2

2 FA groups, 3 carbons, 1 phosphate group with a polar head group

Question 3

What is unique about cardiolipin?

Answer 3

Has 2 phosphate groups with 4 FA groups (total, 2 per a P)

Question 4

Plasmalogens

Answer 4

Synthesized in peroxisomes. Have alkenyl alcohol attached via ether linkage

Question 5

Sphingomyelin

Answer 5

Sphingosine + acyl-CoA+PC

Question 6

Sphingosine

Answer 6

Singaling molecule. Only the anime plus a saturated FA

Question 7

Glycoshingolipid composition

Answer 7

shingosine, FA in amide linkage, 1 or more carb derived structures

Question 8

2 sources of glycerol-3-phosphate

Answer 8

Liver (glycerol) and adipose tissue (dihydroxyacetone phosphate)

Question 9

Where is glycerol kinase primarily expressed?

Answer 9

Liver and intestine

Question 10

TAGS from liver fate

Answer 10

packaged/secreted as VLDL

Question 11

TAGS from adipose fate

Answer 11

stored in lipid droplets of adipocytes

Question 12

TAGs from intestine fate

Answer 12

Packaged as chylomircons for circulation

Question 13

What tissue does not synthesize phsopholipids?

Answer 13

RBCs

Question 14

From what are all glycerophospholipids derived from?

Answer 14

phosphatidic acid

Question 15

What type of synthesis is DAG used for?

Answer 15

PC and PE

Question 16

How is phosphatidic acid activate for cardiolipin and PI synthesis?

Answer 16

Addition of CTP (into CDP-DAG)

Question 17

Dipalmitoylphophatidylcholine

Answer 17

2 palmitic acids. Is an important component of lung surfactant

Question 18

Components of PAF

Answer 18

1 alkyl ether, 1 acetylglyercol, 1phosphocholine

Question 19

Cerebroside components

Answer 19

ceramide + 1 carb

Question 20

Globoside

Answer 20

Ceramide + 2 or more carbs

Question 21

Ganglioside

Answer 21

Globoside+ 1 or more sialic acids (aka NANAs)

Question 22

Tay-Sachs

Answer 22

Sphingolipidose. Deficient in beta-hexosaminidase. Gm2 accumulation. SS: blindness, seizures, cherry-red macula

Question 23

Gaucher

Answer 23

Most common sphinolipidose. deficient in glucocerebrosidase. Glucocerebroside accumulation. Hepatosplenomegaly, long bone osteoporosis, severe neurological signs.

Question 24

Fabry

Answer 24

Sphingolipidose. Deficient in alpha-galactosidase A. Accumulation of trihexosylceramide. SS: angiokeratoma, renal damage, x-linked**

Question 25

Krabbe

Answer 25

Sphingolipidose. Deficient in galactosylceramidase. Accumulation of galactosylceramide and galactosylsphingosine. SS: infantile onset, peripheral neuropathy, loss of myelin

Question 26

Niemann-Pick

Answer 26

Sphingolipidose. Deficient in sphingomyelinase. Accumulation of sphingomyelin. SS: neuropathic/non-neuropathic forms, heptaoslpenomegaly, foamy cells in bone marrow