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

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

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
Volume30
Issue number4
DOIs
Publication statusPublished - 1 Apr 2008

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Keywords

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

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