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

doi:10.1007/s10529-007-9595-z

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

Instituto de Tecnologia Química e Biológica António Xavier (ITQB)

Research output: Contribution to journal › Article

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 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.

Original language	English
Pages (from-to)	603-610
Number of pages	8
Journal	Biotechnology Letters
Volume	30
Issue number	4
DOIs	https://doi.org/10.1007/s10529-007-9595-z
Publication status	Published - 1 Apr 2008

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Keywords

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

Cite this

Gomes, C. N., Palma, A. S., Almeida, R., Regalla, M., McCluskey, L. F., Trojanowski, J. Q., & Costa, J. (2008). Establishment of a cell model of ALS disease: Golgi apparatus disruption occurs independently from apoptosis. Biotechnology Letters, 30(4), 603-610. https://doi.org/10.1007/s10529-007-9595-z

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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 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.",

keywords = "Amyotrophic lateral sclerosis, Apoptosis, Golgi apparatus, Human SOD1 mutant, NSC-34 motor neuron cell line",

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T2 - Golgi apparatus disruption occurs independently from apoptosis

AU - Gomes, Catarina N.

AU - Palma, Angelina S.

AU - Almeida, Rui

AU - Regalla, Manuela

AU - McCluskey, Leo F.

AU - Trojanowski, John Q.

AU - Costa, Júlia

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AB - 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.

KW - Amyotrophic lateral sclerosis

KW - Apoptosis

KW - Golgi apparatus

KW - Human SOD1 mutant

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10.1007/s10529-007-9595-z

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