Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
13 Cards in this Set
- Front
- Back
|
Where are sphingolipids generally found?
|
In membranes.
|
|
|
All sphingolipids are derived from what?
|
Sphingosine, an amino alcohol made from serine and palmitoyl CoA.
|
|
|
All sphingolipid diseases are caused by what?
|
An inability to degrade a specific sphingolipid, generally due to a missing enzyme.
|
|
|
What is the reaction that creats cerimide?
|
Sphingosine + acyl Coa => Ceramide.
Using Acyl Coa transferase |
|
|
What is sphingomyelin, why is it important?
|
Sphingomyelin, made from one of two possible reactions with Ceramide, is an important compound in the nervous system. It is used as a ratio in the testing of amniotic fluid as an indicator of lung maturity.
|
|
|
What is a glycosphingolipid?
|
A sphingolipid with a sugar attached.
|
|
|
What are the 4 kinds of glycosphingolipids?
|
Cerebrosides, sulfatides,Globosides, gangliosides.
|
|
|
How are cerebrosides made?
|
By the reaction of UDP-galactose or UDP-glucose with ceramide.
|
|
|
What is a PAPS and what is it used for?
|
they transfer a sulfate to a galactocerebroside to create a sulfatide.
|
|
|
What defect is responisble for Gaucher's disease?
|
A lack of the enzyme glucocerebrosidase leading to an excess of glucocerebroside in the brain, liver, lungs, spleen and kidneys.
|
|
|
Why is Gauchers diseas called an orphan drug disease?
|
The cost of the treatment, nearly a half a million dollars a year, is too much for most people to pay.
|
|
|
What defect is present in Tay sachs disease?
|
Is an inabillity to break down the ganglioside GM2. This is due to a deffective Hexosaminidase A enzyme. There is no cure.
|
|
|
What is the general structure of a ganglioside?
|
Acidic ceramide oligosaccharides and sialic acid (Multiple sugars + NANA + ceramide
|
