Altered macroautophagy in the spinal cord of S0D1 mutant mice

被引：157

作者：

Li, Liang ^{[1
,2
]}

Zhang, Xiaojie ^{[1
,2
]}

Le, Weidong ^{[1
,2
,3
]}

机构：

[1] Shanghai Jiao Tong Univ, Sch Med, Ruijin Hosp, Inst Neurol, Shanghai 200025, Peoples R China

[2] Chinese Acad Sci, Shanghai Inst Biol Sci, Shanghai, Peoples R China

[3] Jiao Tong Univ, Sch Med, Ruijin Hosp, Inst Neurol, Shanghai 200030, Peoples R China

来源：

AUTOPHAGY | 2008年 / 4卷 / 03期

基金：

中国国家自然科学基金;

关键词：

amyotrophic lateral sclerosis; SOD1(G93A); macroautophagy; transgenic mice; motor neurons;

D O I：

10.4161/auto.5524

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease caused by selective loss of motor neurons (MNs). About 20% familial cases of ALS (fALS) carried the Cu, Zn-superoxide dismutase (SOD1) gene mutation, which plays a crucial role in the pathogenesis of fALS. There is evidence suggesting that macro-autophagy can degrade mutated SOD1 in vitro. To investigate whether the mutant SOD1 can induce macroautophagy in vivo, we examined the LC3 processing in spinal cord and the activation status of macroautophagy in MNs of SOD1(G93A) transgenic mice at different stages. Our data demonstrated that autophagy was activated in spinal cord of SOD1(G93A) Mice indicating a possible role of macroautophagy in the pathogenesis of ALS.

引用

收藏

页码：290 / 293

页数：4

相关论文

共 21 条

[1] Current clinical trials in amyotrophic lateral sclerosis [J].

Bhatt, Jaydeep M. ;

Gordon, Paul H. .

EXPERT OPINION ON INVESTIGATIONAL DRUGS, 2007, 16 (08) :1197-1207

[2]

Boland Barry, 2006, Molecular Aspects of Medicine, V27, P503, DOI 10.1016/j.mam.2006.08.009

[3] Ethnic variation in the incidence of ALS - A systematic review [J].

Cronin, Simon ;

Hardiman, Orla ;

Traynor, Bryan J. .

NEUROLOGY, 2007, 68 (13) :1002-1007

[4] SUMO-1 modification increases human SOD1 stability and aggregation [J].

Fei, Erkang ;

Jia, Nali ;

Yan, Ming ;

Ying, Zheng ;

Sun, Qiang ;

Wang, Hongfeng ;

Zhang, Tao ;

Ma, Xiaochuan ;

Ding, Husheng ;

Yao, Xuebiao ;

Shi, Yunyu ;

Wang, Guanghui .

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 2006, 347 (02) :406-412

[5] Atg9 trafficking in autophagy-related pathways [J].

He, Congcong ;

Klionsky, Daniel J. .

AUTOPHAGY, 2007, 3 (03) :271-274

[6] Degradation of amyotrophic lateral sclerosis-linked mutant Cu,Zn-superoxide dismutase proteins by macroautophagy and the proteasome [J].

Kabuta, Tomohiro ;

Suzuki, Yasuyuki ;

Wada, Keiji .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2006, 281 (41) :30524-30533

[7] A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice [J].

Kieran, D ;

Hafezparast, M ;

Bohnert, S ;

Dick, JRT ;

Martin, J ;

Schiavo, G ;

Fisher, EMC ;

Greensmith, L .

JOURNAL OF CELL BIOLOGY, 2005, 169 (04) :561-567

[8] LC3, an autophagosome marker, can be incorporated into protein aggregates independent of autophagy [J].

Kuma, Akiko ;

Matsui, Makoto ;

Mizushima, Noboru .

AUTOPHAGY, 2007, 3 (04) :323-328

[9] The autophagic and endocytic pathways converge at the nascent autophagic vacuoles [J].

Liou, W ;

Geuze, HJ ;

Geelen, MJH ;

Slot, JW .

JOURNAL OF CELL BIOLOGY, 1997, 136 (01) :61-70

[10] Increased autophagy in transgenic mice with a G93A mutant SOD1 gene [J].

Morimoto, Nobutoshi ;

Nagai, Makiko ;

Ohta, Yasuyuki ;

Miyazaki, Kazunori ;

Kurata, Tomoko ;

Morimoto, Mizuki ;

Murakami, Tetsuro ;

Takehisa, Yasushi ;

Ikeda, Yoshio ;

Kamiya, Tatsushi ;

Abe, Koji .

BRAIN RESEARCH, 2007, 1167 :112-117