Document Detail


Clonal Rett Syndrome cell lines to test compounds for activation of wild-type MeCP2 expression.
MedLine Citation:
PMID:  21840716     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Rett Syndrome is an X-linked progressive neurological disorder caused by inactivation of one allele of the MECP2 gene. There are no curative treatments, and activation of wild-type MECP2 expression is one strategy for stabilizing or reversing the disease. We isolated fibroblast clones that express exclusively either the wild-type or a 32-bp-deletion mutant form of MECP2. We developed a sensitive assay for measuring wild-type MECP2 mRNA levels and tested small molecule epigenetic activators for their ability to activate gene expression. Although our pilot screen did not identify activators of MECP2 expression, it established the value of using clonal cells and defined challenges that must be overcome.
Authors:
Dongbo Yu; Fuminori Sakurai; David R Corey
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2011-07-23
Journal Detail:
Title:  Bioorganic & medicinal chemistry letters     Volume:  21     ISSN:  1464-3405     ISO Abbreviation:  Bioorg. Med. Chem. Lett.     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-08-22     Completed Date:  2011-12-19     Revised Date:  2014-09-17    
Medline Journal Info:
Nlm Unique ID:  9107377     Medline TA:  Bioorg Med Chem Lett     Country:  England    
Other Details:
Languages:  eng     Pagination:  5202-5     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 Elsevier Ltd. All rights reserved.
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MeSH Terms
Descriptor/Qualifier:
Clone Cells / drug effects
Epigenesis, Genetic / drug effects,  genetics
Fibroblasts / drug effects
Gene Expression Profiling
Humans
Methyl-CpG-Binding Protein 2 / genetics*,  metabolism
Molecular Structure
Mutation
Organic Chemicals / chemistry,  pharmacology*
Polymerase Chain Reaction
RNA, Messenger / drug effects,  genetics
Rett Syndrome / genetics,  pathology*
Stereoisomerism
Grant Support
ID/Acronym/Agency:
NIGMS 77253//PHS HHS; R01 GM077253/GM/NIGMS NIH HHS; R01 GM077253-04/GM/NIGMS NIH HHS
Chemical
Reg. No./Substance:
0/Methyl-CpG-Binding Protein 2; 0/Organic Chemicals; 0/RNA, Messenger
Comments/Corrections

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