Ectopic axon hillock-associated neurite growth is maintained in metabolically reversed swainsonine-induced neuronal storage disease

Steven U. Walkley, S. Wurzelmann, D. A. Siegel

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

An experimentally induced and reversible model of a neuronal storage disease, swainsonine-induced feline α-mannosidosis, has been used to study the modifiability of ectopic, axon hillock-associated neurites and their new synaptic contacts. Earlier studies have fully documented that a variety of neuronal storage disorders are characterized by such changes in neuronal geometry and connectivity. Swainsonine administration was ended after 6 months of continuous treatment which had resulted in characteristic signs of α-mannosidosis. Studies of this animal 6 months after reversal showed that even though neuronal vacuolation and other CNS changes essentially normalized, ectopic neurites and their synaptic connections were still present and appeared similar to those of another animal which had been treated with swainsonine for the entire 12-month period. These results suggest that once initiated during the disease process, ectopic axon hillock-associated dendrites become an integral part of the soma-dendritic domain of affected neurons and may not be reversible. These findings may have relevance for current attempts to devise therapies involving enzyme replacement for individuals with inherited neuronal storage disease.

Original languageEnglish (US)
Pages (from-to)89-96
Number of pages8
JournalBrain Research
Volume410
Issue number1
DOIs
StatePublished - Apr 28 1987

Fingerprint

Swainsonine
Mannosidase Deficiency Diseases
Neurites
Growth
Enzyme Replacement Therapy
Felidae
Carisoprodol
Dendrites
Neurons
Axon Initial Segment
Therapeutics

Keywords

  • Enzyme replacement therapy
  • Lysosome
  • Neurite
  • Plasticity
  • Storage disease
  • Swainsonine

ASJC Scopus subject areas

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

Cite this

Ectopic axon hillock-associated neurite growth is maintained in metabolically reversed swainsonine-induced neuronal storage disease. / Walkley, Steven U.; Wurzelmann, S.; Siegel, D. A.

In: Brain Research, Vol. 410, No. 1, 28.04.1987, p. 89-96.

Research output: Contribution to journalArticle

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