Complex Lipids in Health & Disease - IN Flashcards

if nothing else, know the last 9 slides

1
Q

Are Glycerophospholipids Hydrophilic, hydrophobic or amphipathic

A

amphipathic - polar head group attached to DAG

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2
Q

what are the major glycerophospholipids?

A

phosphatidylcholine, phosphatidylserine, phosphatidylethanolamine

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3
Q

what is cardiolipin?

A

a glycerophospholipid found in the inner mitochondrial membrane, supports the structures and functions of certain ETS complexes

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4
Q

what is the simplest glycerophospholipid

A

phosphatidic acid

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5
Q

what is sphingomyelin

A

major component of RBC membrane, the only sphingophospholipid in humans, also present in myelin sheath

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6
Q

what causes respiratory distress syndrome

A

deficiency in surfactant

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7
Q

what is the major component of surfactant

A

DPPC - dipalmitoyl-phosphatidylcholine, produced and secreted from type 2 pneumocytes, reduces alveolar surface tension

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8
Q

what are fatty acids used for in complex lipids

A

synthesis of triacylglycerols for energy storage and phospholipids and sphingolipids for cell membranes

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9
Q

what are glycerophospholipids synthesized from

A

glycerol-3-phosphate and fatty acyl CoA via phosphatidic acid

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10
Q

what are sphingolipids synthesized from

A

sphingosine, derived from serine and palmitoyl CoA

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11
Q

how are phospholipids degraded

A

specific phospholipases….duh!

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12
Q

what are sphingolipidoses

A

lysosomal lipid storage defects based on genetic defects in specific degradation enzymes

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13
Q

what are the ether glycerophospholipids

A

plasmalogen, phosphatidylserine, phosphatidylcholine

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14
Q

what are the subtypes of glycolipids

A

galactocereBROside - found in central and peripheral nervous tissue enriched in myelin sheath
globoside and gangliosides - ganglion cells and nerve endings in CNS

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15
Q

where in the cell does sphingolipid synthesis occur

A

mostly in golgi with sequential addition of monomers by glycosyl transferases

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16
Q

where are choline (PC and SM) containing phospholipids posted up?

A

phosphatidylcholine and sphingomyelin are primarily in the outer leaflet of the membranes

17
Q

where are the glycolipids primarily squatted?

A

outer leaflet

18
Q

where are amine containing phospholipids (PE and PS)

A

phosphatidylethanolamine and phosphatidylserine are primarily in the inner leaflet of membranes

19
Q

what is the structure/function of lipid rafts?

A

specialized cholesterol, sphingolipid, and glycolipid rich domains that regulate activity of membrane enzymes and receptors

20
Q

how are glycerophospholipids degraded

A

phospholipases A1 and A2 cleave off fatty acids at C1 and C2
Phospholipase C cleaves off phosphorylated head group yielding DAG
Phospholipase D cleaves off head group leaving phosphatidic acid

21
Q

How do you treat Gaucher?

A

gene therapy, bone marrow transplant, substrate reduction therapy

22
Q

what are the key steps of plasmalogen synthesis

A

start point is dihydroxyacetone and at the end you get the alkenyl group double bonded in an ether linkage configuration

23
Q

what accumulates in Tay-Sachs, and due to what enzyme deficiency?

A

Gangliosides, B-Hexosaminidase A

24
Q

what accumulates in GM1 Gangliosidosis, and due to what enzyme deficiency?

A

gangliosides & keratan sulfate, B-galactosidase

25
Q

what accumulates in Gaucher, and due to what enzyme deficiency?

A

glucocerebrosides, B-glucosidase

26
Q

what accumulates in Metachromatic Leukodystrophy, and due to what enzyme deficiency?

A

sulfatides, Arylsulfatase A

27
Q

what accumulates in Krabbe Disease, and due to what enzyme deficiency?

A

galactocerebrosides, B-galactosidase

28
Q

what accumulates in Sandhoff, and due to what enzyme deficiency?

A

GM2 & globosides, B-hexosaminidase

29
Q

what accumulates in Fabry, and due to what enzyme deficiency?

A

globosides, a-galactosidase

30
Q

what accumulates in Niemann-Pick, and due to what enzyme deficiency?

A

sphingomyelin, sphingomyelinase

31
Q

what accumulates in Farber, and due to what enzyme deficiency?

A

ceramide, ceraminidase