期刊
BRAIN RESEARCH
卷 1263, 期 -, 页码 122-133出版社
ELSEVIER
DOI: 10.1016/j.brainres.2009.01.041
关键词
NSC-34 motor neuron cell line; Sporadic Amyotrophic Lateral Sclerosis; Cerebrospinal Fluid; Immunocytochemistry; Western blotting
资金
- The Council for Scientific and Industrial Research (CSIR), Govt of India
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. (C) 2009 Elsevier B.V. All rights reserved.
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