Complex Lipids in Health & Disease - IN Flashcards
if nothing else, know the last 9 slides
Are Glycerophospholipids Hydrophilic, hydrophobic or amphipathic
amphipathic - polar head group attached to DAG
what are the major glycerophospholipids?
phosphatidylcholine, phosphatidylserine, phosphatidylethanolamine
what is cardiolipin?
a glycerophospholipid found in the inner mitochondrial membrane, supports the structures and functions of certain ETS complexes
what is the simplest glycerophospholipid
phosphatidic acid
what is sphingomyelin
major component of RBC membrane, the only sphingophospholipid in humans, also present in myelin sheath
what causes respiratory distress syndrome
deficiency in surfactant
what is the major component of surfactant
DPPC - dipalmitoyl-phosphatidylcholine, produced and secreted from type 2 pneumocytes, reduces alveolar surface tension
what are fatty acids used for in complex lipids
synthesis of triacylglycerols for energy storage and phospholipids and sphingolipids for cell membranes
what are glycerophospholipids synthesized from
glycerol-3-phosphate and fatty acyl CoA via phosphatidic acid
what are sphingolipids synthesized from
sphingosine, derived from serine and palmitoyl CoA
how are phospholipids degraded
specific phospholipases….duh!
what are sphingolipidoses
lysosomal lipid storage defects based on genetic defects in specific degradation enzymes
what are the ether glycerophospholipids
plasmalogen, phosphatidylserine, phosphatidylcholine
what are the subtypes of glycolipids
galactocereBROside - found in central and peripheral nervous tissue enriched in myelin sheath
globoside and gangliosides - ganglion cells and nerve endings in CNS
where in the cell does sphingolipid synthesis occur
mostly in golgi with sequential addition of monomers by glycosyl transferases
where are choline (PC and SM) containing phospholipids posted up?
phosphatidylcholine and sphingomyelin are primarily in the outer leaflet of the membranes
where are the glycolipids primarily squatted?
outer leaflet
where are amine containing phospholipids (PE and PS)
phosphatidylethanolamine and phosphatidylserine are primarily in the inner leaflet of membranes
what is the structure/function of lipid rafts?
specialized cholesterol, sphingolipid, and glycolipid rich domains that regulate activity of membrane enzymes and receptors
how are glycerophospholipids degraded
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
How do you treat Gaucher?
gene therapy, bone marrow transplant, substrate reduction therapy
what are the key steps of plasmalogen synthesis
start point is dihydroxyacetone and at the end you get the alkenyl group double bonded in an ether linkage configuration
what accumulates in Tay-Sachs, and due to what enzyme deficiency?
Gangliosides, B-Hexosaminidase A
what accumulates in GM1 Gangliosidosis, and due to what enzyme deficiency?
gangliosides & keratan sulfate, B-galactosidase
what accumulates in Gaucher, and due to what enzyme deficiency?
glucocerebrosides, B-glucosidase
what accumulates in Metachromatic Leukodystrophy, and due to what enzyme deficiency?
sulfatides, Arylsulfatase A
what accumulates in Krabbe Disease, and due to what enzyme deficiency?
galactocerebrosides, B-galactosidase
what accumulates in Sandhoff, and due to what enzyme deficiency?
GM2 & globosides, B-hexosaminidase
what accumulates in Fabry, and due to what enzyme deficiency?
globosides, a-galactosidase
what accumulates in Niemann-Pick, and due to what enzyme deficiency?
sphingomyelin, sphingomyelinase
what accumulates in Farber, and due to what enzyme deficiency?
ceramide, ceraminidase
