Pulm/Renal - Biochemistry - Phospholipids, Sphingolipids, Eicosanoids; the Urea Cycle: Hyperammonemias; Nucleotide Metabolism Flashcards
Name three subtypes of phospholipid.
Phosphoglycerides
Plasmalogens
Sphingolipids
Describe the differences in chemical structure between the following phospholipids:
Phosphoglycerides
Plasmalogens
Sphingolipids
Phosphoglycerides - polar head + 2 esterified fatty acids
Plasmalogens - polar head + 1 esterified fatty acid + 1 etherified fatty acid
Sphingolipids - polar head + 1 ceramide (amide-bonded fatty acid) + 1 sphingosine (enol attachment)

What are the two main types of sphingolipid?
- Sphingomyelin
- Glycolipids (e.g. glucocerebroside, gangliosides, etc.)
Sphingomyelin is basically a phospholipid (specifically, a sphingolipid) with what polar head?
Phosphocholine
What are the basic steps of phosphoglyceride synthesis?
DHAP or glycerol –>
glyceral 3-phosphate –>
activated fatty acic groups added to C1 and C2 –>
head group attached to 3-phosphate

What are the three tissues that are major producers of phosphoglycerides?
Liver
Intestinal epithelium
Lungs (for surfactant)
What are the two major components of pulmonary surfactant?
Surfactant is __% lipid and __% protein.
Dipalmitoylphosphatidylcholine (DPPC),
phosphatidylglycerol;
90, 10
You need to determine fetal surfactant production. Where do you get a sample?
A lecithin:sphingomyelin reading of 1.2 indicates what?
(Note: lecithin is an old term for phosphatidylcholine)
The amniotic fluid;
immature production (<2.0)
What substance should be given to mothers going into preterm labor to reduce their child’s risk of respiratory distress syndrome?
Glucocorticoids
What effect does surfactant have on the alveolus?
Decreased surface tension / elastance;
increased compliance
What is a normal lecithin:sphingomyelin reading for mature lungs?
(Note: lecithin is an old term for phosphatidylcholine)
2:0 - 2:5
Lecithin:sphingomyelin levels are useful in measuring lung maturity.
Lecithin is another name for what substance?
Phosphatidylcholine
Besides their structural role in membranes, what role do plasmalogens (a type of phospholipid) have in cellular protection?
They act as ‘scavengers’ for free radicals, sparing nearby fatty acids and other structures
The nomenclature of plasmalogens is similar to phosphoglycerides.
Describe any differences, using phosphatidylcholine as an example phosphoglyceride.
Phosphoglyceride: Phosphatidylcholine
Plasmalogen: Phosphatidalcholine
Where does plasmalogen (a type of phospholipid) synthesis occur?
What disorder results when peroxisome biogenesis is inhibited?
Peroxisomes;
Zellweger syndrome (death usually occurs within 6 mo. - 1 year following birth)
What enzyme cleaves the C-1 esterified fatty acid from a phosphoglyceride?
What enzyme cleaves the C-2 esterified fatty acid from a phosphoglyceride?
What enzyme cleaves the C-3 phosphate group from a phosphoglyceride?

Phospholipase A1;
phospholipase A2;
phospholipase C

What enzyme cleaves the C-1 esterified fatty acid from a phosphoglyceride?

Phospholipase A1

What enzyme cleaves the C-2 esterified fatty acid from a phosphoglyceride?

Phospholipase A2

What enzyme cleaves the C-3 phosphate group from a phosphoglyceride?

Phospholipase C

Name the function of each of the following:
Phospholipase A1
Phospholipase A2
Phospholipase C
Cleave the phosphoglyceride C-1 fatty acid from the glycerol back bone
Cleave the phosphoglyceride C-2 fatty acid from the glycerol back bone
Cleave the phosphoglyceride C-3 phosphate group from the glycerol backbone

All sphingolipid synthesis start with a simple ___________.
To create sphingomyelin, add ___________.
To create glucosylcerebroside , add ___________.
To create galactosylcerebroside , add ___________.
Ceramide;
phosphocholine,
glucose,
galactose

Sulfatides are a type of sphingolipid made by the ____________ of galactosylcerebrosides in the CNS oligodendrocytes.
If multiple sugars are added to a ceramide, a ______________ results. Adding ________ acid to this results in a ganglioside.
Sulfation;
globoside,
sialic (N-acetylneuraminic)
Type A Niemann-Pick disease results from dysfunction in the metabolism of what substance?
Type B Niemann-Pick disease results from dysfunction in the metabolism of what substance?
Type C1 Niemann-Pick disease results from dysfunction in the metabolism of what substance?
Type C2 Niemann-Pick disease results from dysfunction in the metabolism of what substance?
Sphingomyelin;
sphingomyelin;
cholesterol;
cholesterol
How does type A Niemann-Pick disease manifest?
How does type B Niemann-Pick disease manifest?
More severe form: sphingomyelin accumulation in liver, CNS, and spleen
–> neurodegeneration/intellectual disability and early death;
less severe form: no significant neurological involvement
–> patients typically survive into adulthood











































