Hexacarbon neuropathy

Griffin, J. W. and Price, D. L. Demyelination in experimental beta, beta -iminodipropionitrile and hexacarbon neuropathies. Evidence for an axonal influence. Lab. Invest. 45 130-141,1981. 

Politis MJ, Pellegrino RG, Spencer PS. 1980. Ultrastructural studies of the dying-back process. 5. Axonal neurofilaments accumulate at sites of 2,5-hexanedione application Evidence for nerve fibre dysfunction in experimental hexacarbon neuropathy. [Abstract] J Neurocytol 9 505-516. 

This hypothesis is best supported in the case of the hexacarbons. Hexacarbons can react both in vitro and in vivo with lysine e-amino moieties of proteins to yield pyrrole adducts, which permit secondary auto-oxidative crosslinking. It has been postulated that pyrrole derivitization and secondary crosslinking of neurofilaments are the initiating events in hexacarbon neuropathy. Although this mechanism has been challenged, many studies support this mechanism for the hexacarbons. However, this hypothesis offers no explanation for the early effects on retrograde transport reported in hexacarbon neuropathy. 

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