Document Detail


Clinical and molecular events in patients with Machado-Joseph disease under lamotrigine therapy.
MedLine Citation:
PMID:  15876340     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: Machado-Joseph disease (MJD)/spinocerebellar ataxia type 3 is an autosomal dominant spinocerebellar degeneration, for which there is no effective treatment.
PATIENTS AND METHODS: This study involved the clinical response of lamotrigine (LTG) on six MJD patients with early truncal ataxia and the effect of LTG on the alteration of ataxin-3 expression in the transformed MJD lymphoblastoid cells.
RESULT: LTG medication was found, on the basis of single leg standing test tandem gait index, to effectively improve gait balance, but did not prove to be effective in the withdrawal period. In Western blot analysis of ataxin-3 in MJD lymphoblastoid cells, extracellular application of LTG, while leaving the normal level of ataxin-3 intact, decreased the expression of mutant ataxin-3 in a dose-related manner.
CONCLUSION: Our results indicated that LTG may have significant benefits in relief of gait disturbance in MJD patients with early ataxia, and may be related to the decreased expression of mutant ataxin-3.
Authors:
C-S Liu; H-M Hsu; W-L Cheng; M Hsieh
Publication Detail:
Type:  Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Acta neurologica Scandinavica     Volume:  111     ISSN:  0001-6314     ISO Abbreviation:  Acta Neurol. Scand.     Publication Date:  2005 Jun 
Date Detail:
Created Date:  2005-05-06     Completed Date:  2005-07-28     Revised Date:  2013-08-21    
Medline Journal Info:
Nlm Unique ID:  0370336     Medline TA:  Acta Neurol Scand     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  385-90     Citation Subset:  IM    
Affiliation:
Vascular and Genomic Research Center, Changhua Christian Hospital, Changhua, Taiwan.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Anticonvulsants / pharmacology,  therapeutic use
Cell Line, Transformed
Dose-Response Relationship, Drug
Down-Regulation / drug effects,  physiology
Female
Gait Ataxia / blood*,  drug therapy*,  physiopathology
Humans
Lymphocytes / metabolism
Machado-Joseph Disease / blood*,  drug therapy*,  physiopathology
Male
Mutation / drug effects,  physiology
Nerve Tissue Proteins / genetics,  metabolism*
Nuclear Proteins
Pilot Projects
Postural Balance / drug effects,  physiology
Repressor Proteins
Stem Cells / metabolism
Treatment Outcome
Triazines / pharmacology*,  therapeutic use
Chemical
Reg. No./Substance:
0/Anticonvulsants; 0/Nerve Tissue Proteins; 0/Nuclear Proteins; 0/Repressor Proteins; 0/Triazines; EC 3.4.22.-/ATXN3 protein, human; U3H27498KS/lamotrigine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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