Alterations in neuron morphology in mucopolysaccharidosis type I - A Golgi study

Steven U. Walkley, M. E. Haskins, R. M. Shull

Research output: Contribution to journalArticle

34 Citations (Scopus)

Abstract

Morphological changes in neurons with inborn defects of the lysosomal hydrolase, α-l-iduronidase, and with concomitant storage of glycosaminoglycans, were evaluated by Golgi staining in two animal models and compared to a similar study of a child with the same disease. Cortical pyramidal neurons in feline mucopolysaccharidosis type I often displayed axon hillock enlargements (meganeurites) and/or ectopic, secondary neuritic processes sprouting from this same region of the cell. The latter structures were prominent and often appeared longer than similar neurites reported in other neuronal storage discases. Although most meganeurites were aspiny, a few were observed which possessed spine-like processes or neurites. Other than these morphological changes in cortical pyramidal neurons, few other cell types displayed abnormalities demonstrable by Golgi impregnation. In the canine model of this disorder, abnormal Golgi-impregnated cortical neurons resembled more closely those seen in human mucopolysaccharidosis. That is, they possessed meganeurites which typically were aspiny in appearance. Ectopic neurite growth was not observed on any Golgi-impregnated neurons in the cases of canine or human mucopolysaccharidosis used in this study. The latter finding given the advanced ages of these cases, is consistent with the view that ectopic neuritogenesis seen in neuronal storage diseases may be subject to a developmental window, albeit one open well beyond the period of early postnatal maturation.

Original languageEnglish (US)
Pages (from-to)611-620
Number of pages10
JournalActa Neuropathologica
Volume75
Issue number6
DOIs
StatePublished - Nov 1988

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Mucopolysaccharidosis I
Neurites
Mucopolysaccharidoses
Pyramidal Cells
Neurons
Canidae
Iduronidase
Chymopapain
Felidae
Hydrolases
Glycosaminoglycans
Spine
Animal Models
Staining and Labeling
Growth

Keywords

  • Dendrite
  • Hurler's disease
  • Mucopolysaccharidosis
  • Neurite growth
  • Neuronal storage disease

ASJC Scopus subject areas

  • Neuroscience(all)
  • Pathology and Forensic Medicine
  • Clinical Neurology

Cite this

Alterations in neuron morphology in mucopolysaccharidosis type I - A Golgi study. / Walkley, Steven U.; Haskins, M. E.; Shull, R. M.

In: Acta Neuropathologica, Vol. 75, No. 6, 11.1988, p. 611-620.

Research output: Contribution to journalArticle

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