Establishment of a cell model of ALS disease

Golgi apparatus disruption occurs independently from apoptosis

Catarina N. Gomes, Angelina S. Palma, Rui Almeida, Manuela Regalla, Leo F. McCluskey, John Q. Trojanowski, Júlia Costa

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18 Citations (Scopus)


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 hSOD1G93A, as an ALS cell model, were constructed. The number of cells with disrupted GA increased from 14% to 34%. Furthermore, NSC-34/hSOD1G93A 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.

Original languageEnglish
Pages (from-to)603-610
Number of pages8
JournalBiotechnology Letters
Issue number4
Publication statusPublished - 1 Apr 2008



  • Amyotrophic lateral sclerosis
  • Apoptosis
  • Golgi apparatus
  • Human SOD1 mutant
  • NSC-34 motor neuron cell line

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