Intro
What types of foods contain very long chain polyunsaturated acids?
Fish, dairy products, vegetable oils, flax seeds, walnuts, etc.
What does siRNA stand for?
Short Inhibitory RNA
In Figure 3 Graph A, what mutation is showing the most AlkCho protein deficiency?
Delta PEX13 and Dealt PEX16
What kind of proteins are involved in the elongation of VLCFAs?
Elovl proteins
What pathway is the precursor to fatty acid synthesis?
Pyruvate Oxidation
What are the two diseases studied in this paper?
Zellweger Syndrome and Acyl-CoA Oxidase-1 Deficiency
How many experiments make up each data point?
Three independent experiments
Name one of the phospholipid classes from Figure 2 Graph B.
PtdEtn, PtsEtn, PtdCho, AlkCho, or PtdSer
Where is PlsEtn abundant in?
Myelin
What does the peroxisome do?
Break down VLC fatty acids and build lipids and ether phospholipids (important for brain function)
How are very long chain polyunsaturated acids defined?
At least 22 carbons long and at least 3 double bonds
Name the two cell types used in this paper.
Fibroblasts and CHO cells
What is Figure 1 Graph D showing?
Comparing the expression of PlsEtn at different Alkenyl chains at sn-1
Abnormalities in peroxisomal metabolism may affect biosynthesis of what? Two possible answers.
Plasmalogen or Bile Acids
What family of disorders is this in?
Leukeodystropies
What are the four unique symptoms of Zellweger Syndrome that are not in the other diseases studied in the paper?
Brain dysfunction, facial dimorphism, hypotonia, and early death
What does LC-ESI-MS/MS stand for?
Liquid Chromatography-Electrospray Ionization-Tandem Mass spectroscopy
In figure 4, cell Z65 is treated with a mutagen. What does this mutagen cause?
Deficiency of peroxisomal matrix protein import
PtdEtn was increased, especially in where?
In peroxisome-deficient cells
Generally speaking, what was analyzed in this study?
Lipid composition of skin fibroblasts
How does accumulation of VLC's affect the brain?
Synthesis of important proteins for brain functioning will be inhibited
Name the method used to determine protein concentration during lipid extraction.
BCA method (Bibinchonic acid method)
Why is it less likely that neither ELOVL 1 or ELOVL 5 are involved in the accumulation of VLC-PUFAs?
The knockdown of these proteins did not reduce the accumulation of VlC-PUFAs to the control level.
In mammals, the sn-1 position of PlsEtn typically contains what type of alcohols?
C16, C18:0, or C18:1 fatty alcohols
What is the title of this research paper?
Very-Long-Chain Polyunsaturated Fatty Acids Accumulate in Phosphatidylcholine of Fibroblasts from Patients with Zellweger Syndrome and Acyl-CoA Oxidase1 Deficiency