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

Keywords

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

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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",

author = "Gomes, {Catarina N.} and Palma, {Angelina S.} and Rui Almeida and Manuela Regalla and McCluskey, {Leo F.} and Trojanowski, {John Q.} and J{\'u}lia Costa",

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T1 - Establishment of a cell model of ALS disease

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

PY - 2008/4/1

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N2 - 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.

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

KW - NSC-34 motor neuron cell line

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