Journal ArticleDOI
Redox proteomics analysis of oxidatively modified proteins in G93A-SOD1 transgenic mice--a model of familial amyotrophic lateral sclerosis.
H. Fai Poon,Kenneth Hensley,Kenneth Hensley,Visith Thongboonkerd,Michael L. Merchant,Bert C. Lynn,William M. Pierce,Jon B. Klein,Vittorio Calabrese,D. Allan Butterfield +9 more
Reads0
Chats0
TLDR
It is suggested that oxidative modification of UCH-L1, TCTP, SOD1, and possibly alphaB-crystallin may play an important role in the neurodegeneration of ALS.About:
This article is published in Free Radical Biology and Medicine.The article was published on 2005-08-15. It has received 146 citations till now. The article focuses on the topics: Neurodegeneration & SOD1.read more
Citations
More filters
Journal ArticleDOI
Oxidative stress in ALS: key role in motor neuron injury and therapeutic target.
Siân C. Barber,Pamela J. Shaw +1 more
TL;DR: There is substantial evidence implicating oxidative stress as a central mechanism by which motor neuron death occurs, along with how oxidative damage triggers or exacerbates other neurodegenerative processes, and the trials of a variety of antioxidants as potential therapies for ALS are reviewed.
Journal ArticleDOI
Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS.
TL;DR: mounting evidence from mice with cell restrictive, repressible or chimeric expression of mutant SOD1 transgenes and bone marrow transplants supports non-neuronal origins of neuroprotection in ALS.
Journal ArticleDOI
Oxidative Stress in Neurodegenerative Diseases
Ewa Niedzielska,Irena Smaga,Maciej Gawlik,Andrzej Moniczewski,Piotr Stankowicz,Joanna Pera,Małgorzata Filip,Małgorzata Filip +7 more
TL;DR: The effects of the drug-induced modulation of oxidative balance, and pharmacotherapeutic strategies for OS reduction are analyzed, and they are explored.
Journal ArticleDOI
Oxidative stress in ALS: a mechanism of neurodegeneration and a therapeutic target
TL;DR: The evidence implicating oxidative stress in ALS pathogenesis is reviewed, how oxidative stress may affect and be affected by other proposed mechanisms of neurodegeneration, and the trials of various anti-oxidants as potential therapies for ALS are reviewed.
Journal ArticleDOI
The proteasomal system.
TL;DR: This review tries to summarize the current knowledge on the proteasome and the ubiquitin-proteasomal system, including the cellular functions of this system.
References
More filters
Journal ArticleDOI
The Ubiquitin System
Avram Hershko,Aaron Ciechanover +1 more
TL;DR: This review discusses recent information on functions and mechanisms of the ubiquitin system and focuses on what the authors know, and would like to know, about the mode of action of ubi...
Journal ArticleDOI
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
TL;DR: Tight genetic linkage between FALS and a gene that encodes a cytosolic, Cu/Zn-binding superoxide dismutase (SOD1), a homodimeric metalloenzyme that catalyzes the dismutation of the toxic superoxide anion O–2 to O2 and H2O2 is reported.
Journal ArticleDOI
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation.
Mark E. Gurney,Haifeng Pu,Arlene Y. Chiu,Mauro C. Dal Canto,Cynthia Y. Polchow,Denise D. Alexander,Jan Caliendo,Afif Hentati,Young W. Kwon,Han Xiang Deng,W. Chen,Ping Zhai,Robert L. Sufit,Teepu Siddique +13 more
TL;DR: In this article, the authors found that mutations of human Cu,Zn superoxide dismutase (SOD) contribute to the pathogenesis of familial amyotrophic lateral sclerosis (ALS).
Journal ArticleDOI
Basic Medical Research Award. The ubiquitin system.
TL;DR: The ubiquitin system plays important roles in the control of numerous processes, including cell-cycle progression, signal transduction, transcriptional regulation, receptor down-regulation, and endocytosis as mentioned in this paper.
Journal ArticleDOI
The ubiquitin pathway in Parkinson's disease
Elisabeth Leroy,Rebecca Boyer,Georg Auburger,Barbara Leube,Gudrun Ulm,Eva Mezey,G Harta,Michael J. Brownstein,Sobhanadditya Jonnalagada,Tanya Chernova,Anindya Dehejia,Christian Lavedan,Thomas Gasser,Peter J. Steinbach,Keith D. Wilkinson,Mihael H. Polymeropoulos +15 more
TL;DR: It is shown that in a German family with Parkinson's disease a missense mutation in the ubiquitin carboxy-terminal hydrolase L1 (UCH-L1) gene causes a partial loss of the catalytic activity of this thiol protease, which could lead to aberrations in the proteolytic pathway and aggregation of proteins.