title: Establishment of a cell model of ALS disease: Golgi apparatus disruption occurs independently from apoptosis.
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 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.
authors.affiliation: Instituto de Tecnologia Química e Biológica, Apartado 127, 2781-901 Oeiras, Portugal.
authors.forename: Catarina
authors.initials: C
authors.lastname: Gomes
authors.forename: Angelina S
authors.initials: AS
authors.lastname: Palma
authors.forename: Rui
authors.initials: R
authors.lastname: Almeida
authors.forename: Manuela
authors.initials: M
authors.lastname: Regalla
authors.forename: Leo F
authors.initials: LF
authors.lastname: McCluskey
authors.forename: John Q
authors.initials: JQ
authors.lastname: Trojanowski
authors.forename: Júlia
authors.initials: J
authors.lastname: Costa
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D000818:Animals; D017209:Apoptosis; D053148:Caspase 3 / Q000378:metabolism; D002460:Cell Line; D002470:Cell Survival; D005434:Flow Cytometry; D006056:Golgi Apparatus / Q000378:metabolism*; D006801:Humans; D053078:Membrane Potential, Mitochondrial; D051379:Mice; D008856:Microscopy, Fluorescence; D009046:Motor Neurons / Q000378:metabolism / Q000473:pathology; D009154:Mutation; D013482:Superoxide Dismutase / Q000235:genetics / Q000378:metabolism; D000072105:Superoxide Dismutase-1
chemical_list: C000606290:SOD1 protein, human; C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1; D053148:Caspase 3