Document Detail


Motor neuronal protection by L-arginine prolongs survival of mutant SOD1 (G93A) ALS mice.
MedLine Citation:
PMID:  19427829     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by progressive paralysis due to motor neuron degeneration. Despite the fact that many different therapeutic strategies have been applied to prevent disease progression, no cure or effective therapy is currently available for ALS. We found that L-arginine protects cultured motor neurons from excitotoxic injury. We also found that L-arginine supplementation both prior to and after the onset of motor neuron degeneration in mtSOD1 (G93A) transgenic ALS mice significantly slowed the progression of neuropathology in lumbar spinal cord, delayed onset of motor dysfunction, and prolonged life span. Moreover, L-arginine treatment was associated with preservation of arginase I activity and neuroprotective polyamines in spinal cord motor neurons. Our findings show that L-arginine has potent in vitro and in vivo neuroprotective properties and may be a candidate for therapeutic trials in ALS.
Authors:
Junghee Lee; Hoon Ryu; Neil W Kowall
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, Non-P.H.S.     Date:  2009-05-08
Journal Detail:
Title:  Biochemical and biophysical research communications     Volume:  384     ISSN:  1090-2104     ISO Abbreviation:  Biochem. Biophys. Res. Commun.     Publication Date:  2009 Jul 
Date Detail:
Created Date:  2009-05-27     Completed Date:  2009-06-19     Revised Date:  2013-06-02    
Medline Journal Info:
Nlm Unique ID:  0372516     Medline TA:  Biochem Biophys Res Commun     Country:  United States    
Other Details:
Languages:  eng     Pagination:  524-9     Citation Subset:  IM    
Affiliation:
Department of Neurology, Boston University School of Medicine, Boston, MA 02118, USA. junghee@bu.edu
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MeSH Terms
Descriptor/Qualifier:
Amino Acid Substitution
Amyotrophic Lateral Sclerosis / drug therapy*
Animals
Arginase / metabolism
Arginine / therapeutic use*
Cytoprotection*
Mice
Mice, Transgenic
Motor Neurons / drug effects*,  immunology,  metabolism
Mutation
Neuroprotective Agents / therapeutic use*
Spermidine / immunology,  metabolism
Superoxide Dismutase / genetics
Grant Support
ID/Acronym/Agency:
NS52724/NS/NINDS NIH HHS; P30 AG013846/AG/NIA NIH HHS; P30 AG013846-09/AG/NIA NIH HHS; P30 AG13846/AG/NIA NIH HHS; R01 NS052724-03/NS/NINDS NIH HHS
Chemical
Reg. No./Substance:
0/Neuroprotective Agents; 124-20-9/Spermidine; 74-79-3/Arginine; EC 1.15.1.-/superoxide dismutase 1; EC 1.15.1.1/Superoxide Dismutase; EC 3.5.3.1/Arginase
Comments/Corrections

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