Metabolomic Analysis of Pelizaeus-Merzbacher Disease
Author Information
Author(s): Wood Paul L, Smith Tara, Pelzer Lindsay, Goodenowe Dayan B
Primary Institution: Phenomenome Discoveries Inc
Hypothesis
What are the lipid changes and metabolic consequences in cellular models of Pelizaeus-Merzbacher disease?
Conclusion
The study found significant decreases in plasmalogens and increased levels of myo-inositol in cells affected by Pelizaeus-Merzbacher disease.
Supporting Evidence
- Ethanolamine plasmalogens were significantly decreased in PMD fibroblasts and lymphocytes.
- Cellular levels of myo-inositol were dramatically increased in PMD cells.
- Uptake studies showed increased rates of myo-inositol uptake by PMD lymphocytes.
Takeaway
This study looked at cells from a disease called Pelizaeus-Merzbacher disease and found that they had less of a certain fat and more of a sugar called myo-inositol, which might be causing problems in the cells.
Methodology
Targeted lipidomic and metabolomic analyses were performed on PMD fibroblasts, lymphocytes, and 158JP oligodendrocytes.
Statistical Information
P-Value
p<0.01
Statistical Significance
p<0.01
Digital Object Identifier (DOI)
Want to read the original?
Access the complete publication on the publisher's website