Myelination in the absence of UDP-galactose:ceramide galactosyl-transferase and fatty acid 2-hydroxylase
2011

Myelination Without Certain Sphingolipids

Sample size: 6 publication Evidence: moderate

Author Information

Author(s): Meixner Marion, Jungnickel Julia, Grothe Claudia, Gieselmann Volkmar, Eckhardt Matthias

Primary Institution: Institute of Biochemistry and Molecular Biology, University of Bonn, Germany

Hypothesis

The presence of HFA-GlcCer and HFA-sphingomyelin in Cgt-/- mice is a functionally important compensatory upregulation that prevents a more severe phenotype.

Conclusion

Compact myelin can be formed with non-hydroxylated sphingomyelin as the predominant sphingolipid, but myelin maintenance is impaired.

Supporting Evidence

  • Fa2h-/-/Cgt-/- mice lack sulfatide and GalCer, but still form compact myelin.
  • The amount of GlcCer in CNS myelin was reduced by more than 80% in Fa2h-/-/Cgt-/- mice compared to Cgt-/- mice.
  • Sphingomyelin was significantly increased in Fa2h-/-/Cgt-/- mice.

Takeaway

Even without certain important fats, the brain can still make a protective covering for nerves, but it doesn't work as well over time.

Methodology

The study involved generating double-deficient mice and analyzing their myelin composition and structure through various biochemical and morphological techniques.

Limitations

Extensive behavioral testing could not be performed due to low availability of age- and gender-matched mice.

Participant Demographics

Mice of various genotypes, specifically Cgt-/- and Fa2h-/-/Cgt-/-.

Statistical Information

P-Value

p<0.05

Statistical Significance

p<0.05

Digital Object Identifier (DOI)

10.1186/1471-2202-12-22

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