Reason for demyelinization?

David N Yemchuk dyemchuk at uoguelph.ca
Wed Mar 29 15:21:56 EST 1995


I recently did a presentation on B-mannosidosis. One of the pathological 
signs for this disease is demyelinization of the white matter. 

B-mannosidosis is essentially a storage disease. Specific B1-4 linkages 
between mannose and N-Acetylglucosamine sugar residues cannot be broken 
due to an enzyme deficiency. Hence, these oligosaccharides are accrued 
into secondary lysosomes. Why then does this hinder the normal myelin 
development of Schwann cells in the PNS and by the oligodendrocytes in 
the CNS? I have a hypothesis of my own, but was interested to hear what 
the general public had to say.

Feel free to e-mail me.
Dave.




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