Abstract
PYRAMIDAL neurones of the human cerebral cortex develop spine-bearing structures (meganeurites) between the cell body and the initial portion of the axon in lysosomal storage diseases characterised by intraneuronal accumulation of gangliosides1. Meganeurites attain enormous proportions and acquire bizarre shapes, suggesting that their development involves restricted reactivation of embryonic growth mechanisms of the neurone. Because outgrowth of neurites is a sign of differentiation in embryonic neurones we have sought evidence for neurite outgrowth from meganeurites in feline GM1-ganglioside storage disease2–4. We report here that in this system meganeurites of mature cortical neurones regularly exhibit extensive secondary outgrowth of neurites.
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