Establishment of a cell model of ALS disease: Golgi apparatus disruption occurs independently from apoptosis |
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| Authors: | Catarina Gomes Angelina S. Palma Rui Almeida Manuela Regalla Leo F. McCluskey John Q. Trojanowski Júlia Costa |
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| Affiliation: | (1) Instituto de Tecnologia Química e Biológica, Apartado 127, 2781-901 Oeiras, Portugal;(2) Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA;(3) Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA;(4) Instituto de Biologia Experimental e Tecnológica, Apartado 12, 2781-901 Oeiras, Portugal |
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| 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/hSOD1G93A cells are a suitable cell model to study GA dysfunction in ALS. |
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| Keywords: | Amyotrophic lateral sclerosis NSC-34 motor neuron cell line Human SOD1 mutant Golgi apparatus Apoptosis |
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