Neuronal pathology in the wobbler mouse brain revealed by in vivo proton magnetic resonance spectroscopy and immunocytochemistry

Erik P. Pioro*, Yang Wang, Joanna K. Moore, Thian C. Ng, Bruce D. Trapp, Bogdan Klinkosz, Hiroshi Mitsumoto

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

28 Scopus citations

Abstract

Proton magnetic resonance spectroscopy (1H-MRS) was used to measure the in vivo signal of N-acetylaspartate (NAA), a putative neuronal marker, in the brain of the mutant wobbler mouse, a model of motor neuron disease. The ratio of NAA to creatine-phosphocreatine, an internal standard, was significantly lower in five affected wobbler mice (0.79 ± 0.05; mean ± s.d.) than in five unaffected littermates (0.98 ± 0.10, p = 0.006). Ubiquitin and phosphorylated heavy neurofilament immunoreactivities were increased in cortical neurons of affected animals. This is the first demonstration of cerebral neuronal pathology in the wobbler mouse, supporting its use as a model of amyotrophic lateral sclerosis. In vivo 1H-MRS and correlative postmortem study of wobbler mouse brain will allow temporal monitoring of neuronal degeneration and responsiveness to neuroprotective pharmacotherapies.

Original languageEnglish (US)
Pages (from-to)3041-3046
Number of pages6
JournalNeuroreport
Volume9
Issue number13
DOIs
StatePublished - Sep 14 1998

Keywords

  • Amyotrophic lateral sclerosis
  • Cerebral cortex
  • H- MRS
  • Immunoreactivity
  • N-acetylaspartate
  • Neurodegeneration
  • Phosphorylated heavy neurofilament
  • Ubiquitin

ASJC Scopus subject areas

  • General Neuroscience

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