Cerebrospinal fluid from sporadic amyotrophic lateral sclerosis patients induces degeneration of a cultured motor neuron cell line

Brain Res. 2009 Mar 31;1263:122-33. doi: 10.1016/j.brainres.2009.01.041. Epub 2009 Feb 3.

Abstract

We investigated the effect of Cerebrospinal Fluid (CSF) from sporadic Amyotrophic Lateral Sclerosis patients (SALS-CSF) on motor neuron-like cells to delineate the pathomechanism of SALS. Exposure of NSC-34 cells to SALS-CSF caused lower viability, reduction in differentiation and enhanced lactate dehydrogenase activity. Additionally, reduced choline acetyl transferase expression alongside intracellular aggregation of phosphorylated neurofilaments was prominently seen. The aggregates were immunopositive for ubiquitin. These findings are comparable to the pathological changes seen in the postmortem tissue of ALS patients. Unlimited supply of NSC-34 cells and their vulnerability to SALS-CSF render them to be a good bioassay system to identify new therapeutic agents conferring protection to motor neurons.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyotrophic Lateral Sclerosis / cerebrospinal fluid*
  • Analysis of Variance
  • Animals
  • Blotting, Western
  • Cell Line, Tumor
  • Cell Survival / physiology
  • Choline O-Acetyltransferase / metabolism
  • Cytoskeleton / metabolism
  • Gene Expression
  • Glycolysis / physiology
  • Humans
  • Mice
  • Microscopy, Confocal
  • Motor Neurons / pathology
  • Motor Neurons / physiology*
  • Nerve Degeneration / physiopathology*
  • Neurogenesis / physiology
  • Phosphorylation
  • Photomicrography
  • Ubiquitin / metabolism

Substances

  • Ubiquitin
  • Choline O-Acetyltransferase