1. What is Niemann Pick Disease (A-B)?
    • It is a defect in shpingomyelin degradation. Sphingomylinase deficiency
    • If A- neuro degeneration and early death
    • If B- Spleen, Liver and Marrow degradation
  2. What is a Glycolipid?
    • It is a Glycosphingolipid. attached via an o-linkage. Plasma membrane outer leaflet. Cell-Cell interaction
    • Cerebrosides- a type of GSL

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  3. What type of Cerebroside is in Glycocalyx?
  4. What are gangliosides?
    What group is added at the end?
    What is needed to add this group?
    • They are the most common Glycosphingolipids. Have strong negative charge
    • NANA (Sialic acid) always on the terminal positions.
    • CMP is needed to add this group
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  5. How botox works?
    • Has NANA (Sialic Acid) at terminus
    • inhibits Ach release--> blocks muscle contraction
  6. Sulfatides, what are they made of?
    • Strong negative charge
    • it is a sulfated galactocerebroside
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  7. where is Glycolipids made?
    • in the Golgi
    • addition of glycosyl monomers (last added is 1st removed)
    • all sugars must be in the UDP state
  8. Sphingolipidosi: Types and enzymes affected.
    • Deficiency of specific lysosomal hydrolases
    • Types:
    • Tay-Sachs - Hexosaminidase A (red maculla)
    • Niemann-Pick- Sphingomyelinase
    • Fabrys- (X-linked) alpha galactosidase
    • Gauchers- B-Glucosulceramidase
  9. Where does the assemble of Phospholpids occur?
    The smooth ER
  10. What are the differences in the two groups of Phospholipids?
    • Group 1: Have a glycerol spin
    • Ex: Phosphatidic acid (the simplest one)- inclues Cardiolipin, plasmologens and Platelet activation factor

    Group 2: Phosphorlipids that contain 18 Carbons and Sphingosine instead of glycerol.
  11. What are the 3 origins of Phasphatidic Acid?
    DHAP, Glycerol-3-P, and Diacylglycerol
  12. What is cardiolipin?
    • It is two phosphatidic acids esterfied together.
    • There are 4 FA tails
    • it is in the inner mitochondrial membrane and causes the membrane to be very impermiable
  13. What is Platelet activating factor (PAF)?
    It is a phosphaglyceride that causes platelets to aggragate
  14. What are are two most abundant Phospholipids?
    Phosphatidylethanolamine and phosphatidylcholine
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