Establishment of a cell model of ALS disease: Golgi apparatus disruption occurs independently from apoptosis.
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.
Gomes
Catarina
C
Instituto de Tecnologia Química e Biológica, Apartado 127, 2781-901 Oeiras, Portugal.
Palma
Angelina S
AS
Almeida
Rui
R
Regalla
Manuela
M
McCluskey
Leo F
LF
Trojanowski
John Q
JQ
Costa
Júlia
J
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
C000606290:SOD1 protein, human; C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1; D053148:Caspase 3