Initiation and growth of ectopic neurites and meganeurites during postnatal cortical development in ganglioside storage disease

Steven U. Walkley, Henry J. Baker, Mario C. Rattazzi

Research output: Contribution to journalArticle

29 Scopus citations

Abstract

The incidence of cortical pyramidal neurons displaying meganeurites or enlarged axon hillocks with ectopic spines and neurites was evaluated developmentally using feline models of GM1 and GM2 gangliosidosis. Results of these studies demonstrated that the onset of ectopic neurite growth occurred after the elaboration of dendrites on cortical pyramidal neurons, and that the time of onset of this renewed dendritogenesis was similar in the two diseases. Initiation and growth of ectopic neurites also correlated in a general way with onset and progression of clinical deterioration in both diseases. In GM1 gangliosidosis there was a greater tendency toward formation of meganeurites, whereas in cats with GM2 gangliosidosis the growth of ectopic axon hillock neurites without meganeurites predominated. At end-stage disease in GM2 gangliosidosis, nearly 90% of pyramidal cells displayed some degree of axon hillock neurite growth as opposed to less than half this number for GM1 gangliosidosis cats at the same age. These data are consistent with the hypothesis that there are two separate driving forces behind these somadendritic abnormalities of pyramidal neurons in the gangliosidoses. Excessive intraneuronal accumulation of storage vacuoles accounts for the formation of meganeurites, whereas some type of intrinsic metabolic defect results in axon hillock neurite growth which in turn offers new surface area for synaptic input. Currently available data indicate that GM2 or GM3 ganglioside, or a closely related metabolic product other than GM1 ganglioside, may be primarily associated with the growth of ectopic dendritic processes on morphologically mature neurons in storage diseases.

Original languageEnglish (US)
Pages (from-to)167-178
Number of pages12
JournalDevelopmental Brain Research
Volume51
Issue number2
DOIs
StatePublished - Feb 1 1990

Keywords

  • Cerebral cortex
  • Dendrite
  • Ganglioside
  • Ganglioside storage disease
  • Neurite growth
  • Pyramidal neuron

ASJC Scopus subject areas

  • Developmental Neuroscience
  • Developmental Biology

Fingerprint Dive into the research topics of 'Initiation and growth of ectopic neurites and meganeurites during postnatal cortical development in ganglioside storage disease'. Together they form a unique fingerprint.

  • Cite this