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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
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
What type of Cerebroside is in Glycocalyx?
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
How botox works?
- Has NANA (Sialic Acid) at terminus
- inhibits Ach release--> blocks muscle contraction
Sulfatides, what are they made of?
- Strong negative charge
- it is a sulfated galactocerebroside
where is Glycolipids made?
- in the Golgi
- addition of glycosyl monomers (last added is 1st removed)
- all sugars must be in the UDP state
Sphingolipidosi: Types and enzymes affected.
- Deficiency of specific lysosomal hydrolases
- Tay-Sachs - Hexosaminidase A (red maculla)
- Niemann-Pick- Sphingomyelinase
- Fabrys- (X-linked) alpha galactosidase
- Gauchers- B-Glucosulceramidase
Where does the assemble of Phospholpids occur?
The smooth ER
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.
What are the 3 origins of Phasphatidic Acid?
DHAP, Glycerol-3-P, and Diacylglycerol
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
What is Platelet activating factor (PAF)?
It is a phosphaglyceride that causes platelets to aggragate
What are are two most abundant Phospholipids?
Phosphatidylethanolamine and phosphatidylcholine
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