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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)
Fabrys- (X-linked) alpha galactosidase
Where does the assemble of Phospholpids occur?
The smooth ER
What are the differences in the two groups of Phospholipids?
: Have a glycerol spin
: 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