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Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by selective degeneration of motor neurons. Mutant superoxide dismutase 1 (SOD1) is often found as aggregates in the cytoplasm in motor neurons of various mouse models and familial ALS patients. The interplay between motor neurons and astrocytes is crucial for disease outcome, but the mechanisms underlying this phenomenon remain unknown. In this study, we investigated whether transient transfection with wild-type and mutant-type SOD1 may lead to amplification of mutant SOD1-mediated toxicity in cortical neurons and astrocytes derived from wild-type and mutant-type (human G93A-SOD1) mice. In transgenic mice expressing either wild- or mutant-type SOD1, we found that green fluorescent protein (GFP)-wtSOD1 was present in the cytoplasm and nuclei of wild-type cortical neurons and astrocytes, whereas GFP-mutant SOD1 was mainly cytoplasmic in wild- and mutant-type cortical neurons and astrocytes. These findings indicate that intracellular propagation of misfolding of GFP-wt or mtSOD1 are possible mediators of toxic processes involved in initiating mislocalization and aggregation. Here, we provide evidence that cytoplasmic aggregates induce apoptosis in G93A-SOD1 mouse cortical neurons and astrocytes and that the toxicity of mutant SOD1 in astrocytes is similar to the pathological effects of ALS on neurons in vitro. Collectively, our results indicate that mtSOD1 probably interacts with wtSOD1 via an unknown mechanism to produce augmented toxicity and may influence aggregate formation and apoptosis.

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Modulation of SOD1 Subcellular Localization by Transfection with Wild : or Mutant-type SOD1 in Primary Neuron and Astrocyte Cultures from ALS Mice Do-Yeon Lee, Gye Sun Jeon, Yu-mi Shim, Seung-Yong Seong, Kwang-Woo Lee, Jung-Joon Sung pp.226-234
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Control of Mitochondrial Dynamics by Fas-induced Caspase-8 Activation in Hippocampal Neurons Hyo Min Cho, Woong Sun pp.219-225
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참고문헌 (24건) : 자료제공( 네이버학술정보 )

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번호 참고문헌 국회도서관 소장유무
1 Okado-Matsumoto A, Fridovich I (2002) Amyotrophic lateral sclerosis: a proposed mechanism. Proc Natl Acad Sci U S A 99:9010-9014. 미소장
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3 Ilieva H, Polymenidou M, Cleveland DW (2009) Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond. J Cell Biol 187:761-772. 미소장
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5 Nagai M, Re DB, Nagata T, Chalazonitis A, Jessell TM, Wichterle H, Przedborski S (2007) Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons. Nat Neurosci 10:615-622. 미소장
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7 Son M, Cloyd CD, Rothstein JD, Rajendran B, Elliott JL (2003)Aggregate formation in Cu,Zn superoxide dismutase-related proteins. J Biol Chem 278:14331-14336. 미소장
8 Watanabe M, Dykes-Hoberg M, Culotta VC, Price DL, Wong PC, Rothstein JD (2001) Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues. Neurobiol Dis 8:933-941. 미소장
9 Barbeito LH, Pehar M, Cassina P, Vargas MR, Peluffo H, Viera L, Estévez AG, Beckman JS (2004) A role for astrocytes in motor neuron loss in amyotrophic lateral sclerosis. Brain Res Brain Res Rev 47:263-274. 미소장
10 Wang J, Slunt H, Gonzales V, Fromholt D, Coonfield M, Copeland NG, Jenkins NA,Borchelt DR (2003) Copperbinding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature. Hum Mol Genet 12:2753-2764. 미소장
11 Deng HX, Shi Y, Furukawa Y, Zhai H, Fu R, Liu E, Gorrie GH, Khan MS, Hung WY, Bigio EH, Lukas T, Dal Canto MC, O'Halloran TV, Siddique T (2006) Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria. Proc Natl Acad Sci U S A 103:7142-7147. 미소장
12 Chattopadhyay M, Durazo A, Sohn SH, Strong CD, Gralla EB, Whitelegge JP, Valentine JS (2008) Initiation and elongation in fibrillation of ALS-linked superoxide dismutase. Proc Natl Acad Sci U S A 105:18663-18668. 미소장
13 Hilgenberg LG, Smith MA (2007) Preparation of dissociated mouse cortical neuron cultures. J Vis Exp 10:562. 미소장
14 Jeon GS, Nakamura T, Lee JS, Choi WJ, Ahn SW, Lee KW, Sung JJ, Lipton SA (2014) Potential effect of S-nitrosylated protein disulfide isomerase on mutant SOD1 aggregation and neuronal cell death in amyotrophic lateral sclerosis. Mol Neurobiol 49:796-807. 미소장
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16 Andrus PK, Fleck TJ, Gurney ME, Hall ED (1998) Protein oxidative damage in a transgenic mouse model of familial amyotrophic lateral sclerosis. J Neurochem 71:2041-2048. 미소장
17 Khare SD, Caplow M, Dokholyan NV (2004) The rate and equilibrium constants for a multistep reaction sequence for the aggregation of superoxide dismutase in amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A 101:15094-15099. 미소장
18 Rakhit R, Crow JP, Lepock JR, Kondejewski LH, Cashman NR, Chakrabartty A (2004) Monomeric Cu,Zn-superoxide dismutase is a common misfolding intermediate in the oxidation models of sporadic and familial amyotrophic lateral sclerosis. J Biol Chem 279:15499-15504. 미소장
19 Ezzi SA, Urushitani M, Julien JP (2007) Wild-type superoxide dismutase acquires binding and toxic properties of ALSlinked mutant forms through oxidation. J Neurochem 102:170-178. 미소장
20 Wilcox KC, Zhou L, Jordon JK, Huang Y, Yu Y, Redler RL, Chen X, Caplow M, Dokholyan NV (2009) Modifications of superoxide dismutase (SOD1) in human erythrocytes: a possible role in amyotrophic lateral sclerosis. J Biol Chem 284:13940-13947. 미소장
21 Chia R, Tattum MH, Jones S, Collinge J, Fisher EM, Jackson GS (2010) Superoxide dismutase 1 and tgSOD1 mouse spinal cord seed fibrils, suggesting a propagative cell death mechanism in amyotrophic lateral sclerosis. PLoS One 5:e10627. 미소장
22 Münch C, O’Brien J, Bertolotti A (2011) Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells. Proc Natl Acad Sci U S A 108:3548-3553. 미소장
23 Ravits J, Laurie P, Fan Y, Moore DH (2007) Implications of ALS focality: rostral-caudal distribution of lower motor neuron loss postmortem. Neurology 68:1576-1582. 미소장
24 Pokrishevsky E, Grad LI, Yousefi M, Wang J, Mackenzie IR, Cashman NR (2012) Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis. PLoS One 7:e35050. 미소장

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