Cerebroside

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General formula of the cerebrosides, the remainder R is a hexose .
β- D- galactosylceramide, a galactocerebroside. The radical R stands for the hydrocarbon chain of a fatty acid.

Cerebrosides (also cerebrosides in German-speaking countries ) are a group of glycosphingolipids that are mainly found in nerve tissue . There they occur particularly in the myelin .

Structurally, the cerebrosides consist of a ceramide to which a monohexose, mostly galactose (in the brain ) or glucose ( liver and spleen ), is bound glycosidically . A distinction is made accordingly between galactocerebrosides and glucocerebrosides . In contrast to the cerebrosides, globosides have more than one neutral monosaccharide head group.

Depending on the fatty acid that occurs, a distinction is made between different subspecies of cerebrosides:

fatty acid designation
Lignoceric acid Cerasin
Hydroxylignoceric or
cerebronic acid
Cerebron
Nervonic acid Nervon
Oxynervonic acid Oxynervon

A special case of the galactocerebrosides are the sulfatides , they are β-sulfogalactocerebrosides.

Diseases

In Gaucher's disease , the patient lacks an enzyme , glucocerebrosidase , which splits glucocerebroside into sugar and fat. Thus these accumulate in the body's phagocytes . This leads to an enlarged liver and spleen.

In Krabbe's disease sufferers lack the enzyme β-galactosidase , which splits the galactocerebrosides. The brain is mainly affected.

literature

  • Jeremy M. Berg, John L. Tymoczko, Lubert Stryer : Biochemistry. 6 edition, Spektrum Akademischer Verlag, Heidelberg 2007. ISBN 978-3-8274-1800-5 .
  • Donald Voet, Judith G. Voet: Biochemistry. 3rd edition, John Wiley & Sons, New York 2004. ISBN 0-471-19350-X .
  • Bruce Alberts , Alexander Johnson, Peter Walter, Julian Lewis, Martin Raff, Keith Roberts: Molecular Biology of the Cell , 5th Edition, Taylor & Francis 2007, ISBN 978-0815341062 .