Motor neuronal death in sporadic amyotrophic lateral sclerosis (ALS) is not apoptotic. A comparative study of ALS and chronic aluminium chloride neurotoxicity in New Zealand white rabbits

Neuropathol Appl Neurobiol. 2000 Apr;26(2):150-60. doi: 10.1046/j.1365-2990.2000.026002150.x.

Abstract

Whether diseased motor neurones in sporadic amyotrophic lateral sclerosis (ALS) die via apoptosis is unknown. Because this relates primarily to difficulties in utilizing post-mortem tissue from end-stage disease, motor neurone degeneration in ALS spinal cord was compared with that of a model of a chronic motor neurone degeneration. Degenerating motor neurones in ALS, identified by ubiquitin immunoreactivity, did not demonstrate the morphological characteristics of apoptosis and were not c-Jun immunoreactive or TUNEL positive. A temporal analysis of spinal motor neurone death in the chronic AlCl3 neurotoxicity model of motor neurone degeneration was also undertaken. AlCl3 was administered intracisternally every 4 weeks and, at intervals of 51, 107, 156 and 267 days, evidence of apoptosis was sought by morphology, TUNEL hybridization or DNA laddering. Double-labelling immunostudies were also performed with antibodies to either c-Jun, ubiquitin or high molecular weight neurofilament (NFH) with TUNEL hybridization. Although significant neurone loss was evident, apoptosis was not found. These studies demonstrate a lack of apoptosis in ALS spinal motor neurones and suggest that this observation does not relate to the utilization of post-mortem tissue in which apoptotic neurones may have been lost.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Aged
  • Aluminum Chloride
  • Aluminum Compounds / toxicity*
  • Amyotrophic Lateral Sclerosis / chemically induced*
  • Amyotrophic Lateral Sclerosis / pathology*
  • Animals
  • Antibodies, Monoclonal
  • Apoptosis*
  • Astringents / toxicity*
  • Chlorides / toxicity*
  • DNA Fragmentation
  • Disease Models, Animal
  • Female
  • Humans
  • In Situ Nick-End Labeling
  • Male
  • Middle Aged
  • Motor Neurons / chemistry
  • Motor Neurons / pathology*
  • Neurofilament Proteins / analysis
  • Neurofilament Proteins / immunology
  • Proto-Oncogene Proteins c-jun / analysis
  • Proto-Oncogene Proteins c-jun / immunology
  • Rabbits
  • Spinal Cord / pathology
  • Ubiquitins / analysis
  • Ubiquitins / immunology

Substances

  • Aluminum Compounds
  • Antibodies, Monoclonal
  • Astringents
  • Chlorides
  • Neurofilament Proteins
  • Proto-Oncogene Proteins c-jun
  • Ubiquitins
  • neurofilament protein H
  • Aluminum Chloride