@article{ad2276fa25ee457fbb2968e3284092a4,
title = "Altered macroautophagy in the spinal cord of SOD1 mutant mice",
abstract = "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 macroautophagy 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 SOD1G93A mice indicating a possible role of macroautophagy in the pathogenesis of ALS.",
keywords = "Amyotrophic lateral sclerosis, Macroautophagy, Motor neurons, SOD1, Transgenic mice",
author = "Ang Li and Xiaojie Zhang and Weidong Le",
note = "Funding Information: This study was funded by a research grant Bioscience. from the Science and Technology Commission of Shanghai Municipality (NO. 05DJ14007), and research grants from the Chinese National Sciences Foundation (NO. 30570560 and NO. 30730096). We give special thanks to the Electron Microscope Laboratory of Shanghai Medical College of Fudan University for their technical guidance in electron microscopy. Reference 1. Bhatt JM, Gordon PH. Current clinical trials in amyotrophic lateral sclerosis. Expert Opin Investig Drugs 2007; Landes 16:1197-207. 2. Cronin S, Hardiman O, Traynor BJ. Ethnic variation in the incidence of ALS: A systematic review. Neurology 2007; 68:1002-7. 3. Fei E, Jia N, Yan M, Ying Z, Sun Q, Wang H, Zhang T, Ma X, Ding H, Yao X, Shi Y, Wang G. SUMO-1 modification increases human SOD1 stability and aggregation. Biochem Biophys Res Commun 2006; 347:406-12. 4. Rubinsztein DC. The roles of intracellular protein-degradation pathways in neurodegenera-tion. Nature 2006; 443:780-6. 5. Wootz H, Weber E, Korhonen L, Lindholm D. Altered distribution and levels of cathep-sinD and cystatins in amyotrophic lateral sclerosis transgenic mice: Possible roles in motor {\textcopyright}2008 neuron survival. Neuroscience 2006; 143:419-30. 6. Kabuta T, Suzuki Y, Wada K. Degradation of amyotrophic lateral sclerosis-linked mutant Cu,Zn-superoxide dismutase proteins by macroautophagy and the proteasome. J Biol Chem 2006; 281:30524-33. 7. Yamamoto A, Cremona ML, Rothman JE. Autophagy-mediated clearance of huntingtin aggregates triggered by the insulin-signaling pathway. J Cell Biol 2006; 172:719-31.",
year = "2008",
month = apr,
day = "1",
doi = "10.4161/auto.5524",
language = "English (US)",
volume = "4",
pages = "290--293",
journal = "Autophagy",
issn = "1554-8627",
publisher = "Taylor and Francis Ltd.",
number = "3",
}