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Sphingolipids

Sphingolipids - UTSW

QuestionAnswer
What are the 3 major classes of membrane lipids? Phospholipids (65%), Cholesterol (25%), & Sphingolipids (10%)
What are the 2 types of sphingolipid? Sphingomyelin & Glycosphingolipids
How do sphingomyelin & glycosphingolipids differ structurally? Both have choline & Glycosphingolipids have sugars (glucose & hexose)
How are glycosphingolipids unique? They are amphipathic (hydro philic& phobic)
What is the structure of sphingolipids? sphingosine (18C palmitate) connected by amide (serine) linkage to long chain fatty acid
What are the 3 classes of glycosphingolipids? cerebrosides (1 sugar: galactose (neurons) or glucose (non-neurons)), globosides (linear), gangliosides (branched)
What are first 4 steps of sphingolipid synthesis? 1. Serine + Palmitoyl CoA via Serine palmitoyl transferase *rate limiting*2. 3-ketosphinganine + NADPH via 3-ketosphinganine reductase3. Dihydrosphingosine + RCSCoA via Ceramide synthase4. Dihydroceramide + FAD via Dihydroceramide desaturase
What is the 5th step of sphingolipid synthesis? Branch point: 1. Ceramide + Phosphatidyl Choline via Sphigomyelin synthase leads to sphingomyelin2. Ceramide + UDP Glucose (glycosylation) via glucosylceramide synthase leads to cerebrosides & gangliosides
Where does the 5th step of sphingolipid synthesis occur? Golgi
Where do steps 1-4 of sphingolipid synthesis occur? ER
How does the ceramide in sphingolipid synthesis get from the ER to the Golgi? The ceramide transport protein (CERT)
What biological function do sphingolipids perform in the CNS? They form myelin sheaths (Negatively charged gangliosides in Glial cell membranes)
What are the most abundant gangliosides in myelin? cerebrosides
What biological function do sphingolipids perform in the in non-neuronal cells? Establish asymmetry in lipid composition of membrane & organelles
Why is lipid asymetry useful? Allows the formation of rafts of lipids that have lower phase transition temperature & are stiffer. Membrane proteins associate with rafts
What function of sphingolipids is related to hematology? Glycosphingolipids on the surface of plasma cells form the ABO blood group antigen series
How is a person's blood type determined? The presence or absence of specific sugars attached to gangliosides
Where are sphingolipids destroyed? lysosomes
What are lipid storage diseases? Inherited disorder due to the deficiency/absence of sphingolipid catabolic enzymes causing sphingolipid accumulation
What lipid disease involves the lipid gal-gal-glucosylceramide (ganglioside) due to deficient enzyme alpha-galactosidase? Fabry's
What lipid disease involves the lipid glucosylceramide (cerebroside) due to deficient enzyme beta-glucosidase? Gaucher's
What lipid disease involves the lipid sphingomyelin (sphingolipid) due to deficient enzyme sphingomyelinase? Niemann-Pick
What lipid disease involves the lipid GM2 ganglioside (ganglioside) due to deficient enzyme hexosaminidase A? Tay-Sachs
What are the symptoms of Fabry's? Peripheral neuropathy of hands/feet, angiokeratomas, cardiovascular/renal disease
What are the symptoms of Gaucher's? Hepatosplenomegaly, aseptic neccrosis of femur, bone crises, Gaucher's cells (macrophages that look like crumpled tissue paper)
What are the symptoms of Niemann-Pick? Progressive neurodegeneration,hepatosplenomegaly, cherry-red spot on macula, foam cells
What are the symptoms of Tay-Sachs? Progressive neurodegeneration,developmental delay, cherry-red spot on macula, lysosomes with onion skin
Which lipodoses is the most common? Gaucher's
Which lipodoses are X-linked recessive? Fabry's & Hunter's
Are lipodoses dominant or recessive? recessive
Which lipodoses have hepatosplenomegaly as a symptom? Gaucher's & Niemann-Pick
Which lipodoses have cherry red spot on macula as a symptom? Tay-Sachs & Niemann-Pick
Which lipodoses have progressive neurodegeneration as a symptom? Tay-Sachs & Niemann-Pick
Created by: UTSW1
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