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.
