TY - JOUR
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
Y1 - 2008/4/1
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
UR - http://www.scopus.com/inward/record.url?scp=43149109289&partnerID=8YFLogxK
U2 - 10.1007/s10529-007-9595-z
DO - 10.1007/s10529-007-9595-z
M3 - Article
C2 - 18004513
AN - SCOPUS:43149109289
VL - 30
SP - 603
EP - 610
JO - Biotechnology Letters
JF - Biotechnology Letters
SN - 0141-5492
IS - 4
ER -