Mouse Models with Gene Deletions of Enzymes and Cofactors Involved in Sphingolipid Synthesis and Degradation

  • R. Jennemann
  • H. -J. Gröne
  • H. Wiegandt
  • R. Sandhoff
Reference work entry


Sphingolipids are constituents of the cell membrane. They are believed to play critical roles in many cellular events such as signaling, in modulation of cell adhesion, and as receptor molecules in cell recognition. They may also be involved in cell differentiation, cancer development, and intracellular transport.

The core constituent of sphingolipids is a sphingosine base. Acylation of the aminogroup leads to ceramide. Furthermore, carbohydrates, a phosphate, or phosphorylcholine may be linked to ceramide resulting into glycosphingolipids, ceramide-1-phosphate, and sphingomyelin. Linkage of a phosphate group to sphingosine leads to sphingosine-1-phosphate.

Mouse models with gene deletions of enzymes necessary for sphingolipid synthesis and degradation have been generated. These models provide insights into the cellular functions of sphingolipids.


Gauche Disease Sphingosine Kinase Myelin Associate Glycoprotein Neomycin Resistance Gene Gauche Disease Patient 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

List of Abbreviations:




central nervous system


Cerebroside sulfotransferase


Farber Disease


Gaucher disease


globoid cell leukodystrophy




myelin associated glycoprotein


myelin and lymphocyte protein


myelin basic protein


myelin basic protein positive


Niemann-Pick disease


proteolipid protein


peripheral nervous system


sphingolipid activator proteins


single insertion mutagenesis procedure




Serine palmitoyl-CoA transferase


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© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  • R. Jennemann
  • H. -J. Gröne
  • H. Wiegandt
  • R. Sandhoff

There are no affiliations available

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