Rhm. King et al., AXONAL NEUROFILAMENTOUS ACCUMULATIONS - A COMPARISON BETWEEN HUMAN AND CANINE GIANT AXONAL NEUROPATHY AND 2,5-HD NEUROPATHY, Neuropathology and applied neurobiology, 19(3), 1993, pp. 224-232
The neuropathy produced by the hexacarbon 2,5-hexanedione (2,5-HD) res
embles human and canine inherited giant axonal neuropathy (GAN) in the
presence of giant axonal swellings that contain accumulations of neur
ofilaments. The accumulations are both paranodal and internodal in GAN
and 2,5-HD induced neuropathy. Detailed morphometry on the neurofilam
ents reveals that the changes in human and canine GAN are closely simi
lar and differ from those of 2,5-HD neuropathy, suggesting that the me
chanisms underlying the formation of the axonal neurofilamentous accum
ulations differ between the two conditions. In both human and canine G
AN, the neurofilaments are more closely spaced and are of greater diam
eter than in 2,5-HD neuropathy. The changes in the NF in GAN may be th
e consequence of flattening of the side-arms of the neurofilaments aga
inst the axis of the filaments