Document Detail


In vivo functional brain imaging and a therapeutic trial of l-arginine in MELAS patients.
MedLine Citation:
PMID:  21600268     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
BACKGROUND: Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is the most common type of mitochondrial disease and is characterized by stroke-like episodes (SEs), myopathy, lactic acidosis, diabetes mellitus, hearing-loss and cardiomyopathy. The causal hypotheses for SEs in MELAS presented to date are angiopathy, cytopathy and neuronal hyperexcitability. l-arginine (Arg) has been applied for the therapy in MELAS patients. METHODS: Recent advanced in vivo imaging techniques such as MRI, MRS and PET were applied for evaluating the pathogenesis of SEs (i.e. angiopathy and cytopathy) and monitoring the biochemical effects of l-Arg. RESULTS: Administration of l-Arg to MELAS patients has been successful in reducing neurological symptoms due to acute strokes and preventing recurrences of SEs in the chronic phase. l-Arg has dual pharmaceutical effects on both angiopathy and cytopathy in MELAS. CONCLUSIONS: In vivo functional brain imaging promotes a better understanding of the pathogenesis and potential therapies for MELAS patients. This article is part of a Special Issue entitled Biochemistry of Mitochondria, Life and Intervention 2010.
Authors:
Makoto Yoneda; Masamichi Ikawa; Kenichiro Arakawa; Takashi Kudo; Hirohiko Kimura; Yasuhisa Fujibayashi; Hidehiko Okazawa
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-5-8
Journal Detail:
Title:  Biochimica et biophysica acta     Volume:  -     ISSN:  0006-3002     ISO Abbreviation:  -     Publication Date:  2011 May 
Date Detail:
Created Date:  2011-5-23     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0217513     Medline TA:  Biochim Biophys Acta     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011. Published by Elsevier B.V.
Affiliation:
Department of Neurology, Faculty of Medical Sciences, University of Fukui, Fukui, Japan.
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