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

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.

journal_name

Brain Res

journal_title

Brain research

authors

Vijayalakshmi K,Alladi PA,Sathyaprabha TN,Subramaniam JR,Nalini A,Raju TR

doi

10.1016/j.brainres.2009.01.041

subject

Has Abstract

pub_date

2009-03-31 00:00:00

pages

122-33

eissn

0006-8993

issn

1872-6240

pii

S0006-8993(09)00175-9

journal_volume

1263

pub_type

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