Distribution of ectopic neurite growth and other geometrical distortions of CNS neurons in feline GM2 gangliosidosis

Steven U. Walkley, S. Wurzelmann, M. C. Rattazzi, H. J. Baker

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

Golgi and combined Golgi-electron microscopic (EM) studies were carried out on cats in the terminal stages of GM2 ganglioside storage disease and the resulting data were compared with those from similar studies other neuronal storage diseases in cats, including GM1 gangliosidosis. The results support the view that only limited types of neurons affected by the lysosomal hydrolase deficiency and subsequent intracellular storage have the capacity to sprout new dendritic-like growth processes from their axon hillocks, and that these neurons are essentially the same in all of these diseases studied to date. Golgi studies of CNS tissues from GM2 gangliosidosis cats revealed ectopic neurite growth on pyramidal neurons of cerebral cortex and multipolar cells of amygdala and claustrum, whereas other types of neurons responded to the metabolic defect with aspiny meganeurite formation or somatic enlargement, or appeared normal in terms of soma-dendritic morphology. Combined Golgi-EM studies of cortical pyramidal neurons revealed that ectopic, axon hillock neurites commonly possessed asymmetrical synapses which were similar to those observed in other storage disorders.

Original languageEnglish (US)
Pages (from-to)63-73
Number of pages11
JournalBrain Research
Volume510
Issue number1
DOIs
StatePublished - Feb 26 1990

Fingerprint

GM2 Gangliosidosis
Felidae
Neurites
Pyramidal Cells
Neurons
Cats
Gangliosidoses
Growth
GM1 Gangliosidosis
Cat Diseases
Electrons
Carisoprodol
Hydrolases
Amygdala
Basal Ganglia
Cerebral Cortex
Synapses
Axon Initial Segment

Keywords

  • Dendrite
  • Ganglioside
  • Ganglioside storage disease
  • Neurite growth
  • Neuron
  • Synapse formation

ASJC Scopus subject areas

  • Developmental Biology
  • Molecular Biology
  • Clinical Neurology
  • Neuroscience(all)

Cite this

Distribution of ectopic neurite growth and other geometrical distortions of CNS neurons in feline GM2 gangliosidosis. / Walkley, Steven U.; Wurzelmann, S.; Rattazzi, M. C.; Baker, H. J.

In: Brain Research, Vol. 510, No. 1, 26.02.1990, p. 63-73.

Research output: Contribution to journalArticle

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