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Biotechnol Lett. 2008 Apr;30(4):603-10. Epub 2007 Nov 15.

Establishment of a cell model of ALS disease: Golgi apparatus disruption occurs independently from apoptosis.

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Instituto de Tecnologia Química e Biológica, Apartado 127, 2781-901 Oeiras, Portugal.


The Golgi apparatus (GA) appears disrupted in motor neurons of amyotrophic lateral sclerosis (ALS). Here, mouse motor neuron-like NSC-34 cell lines stably expressing human superoxide dismutase 1 (hSOD1)(wt) and mutant hSOD1(G93A), as an ALS cell model, were constructed. The number of cells with disrupted GA increased from 14% to 34%. Furthermore, NSC-34/hSOD1(G93A) cells showed lower levels of proliferation and differentiation. GA disruption was not caused by apoptosis as determined by several techniques including caspase-3 activation. Similarly, spinal cords from ALS patients did not show caspase-3 activation. Therefore, NSC-34/hSOD1(G93A) cells are a suitable cell model to study GA dysfunction in ALS.

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