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| pubmed-article:16857735 | lifeskim:mentions | umls-concept:C0016719 | lld:lifeskim |
| pubmed-article:16857735 | lifeskim:mentions | umls-concept:C0012854 | lld:lifeskim |
| pubmed-article:16857735 | lifeskim:mentions | umls-concept:C0021467 | lld:lifeskim |
| pubmed-article:16857735 | lifeskim:mentions | umls-concept:C0332281 | lld:lifeskim |
| pubmed-article:16857735 | lifeskim:mentions | umls-concept:C1414899 | lld:lifeskim |
| pubmed-article:16857735 | lifeskim:mentions | umls-concept:C0028736 | lld:lifeskim |
| pubmed-article:16857735 | lifeskim:mentions | umls-concept:C0040649 | lld:lifeskim |
| pubmed-article:16857735 | lifeskim:mentions | umls-concept:C1705914 | lld:lifeskim |
| pubmed-article:16857735 | lifeskim:mentions | umls-concept:C0021469 | lld:lifeskim |
| pubmed-article:16857735 | pubmed:issue | 31 | lld:pubmed |
| pubmed-article:16857735 | pubmed:dateCreated | 2006-8-2 | lld:pubmed |
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| pubmed-article:16857735 | pubmed:abstractText | The DNA abnormality found in 98% of Friedreich's ataxia (FRDA) patients is the unstable hyperexpansion of a GAA.TTC triplet repeat in the first intron of the frataxin gene. Expanded GAA.TTC repeats result in decreased transcription and reduced levels of frataxin protein in affected individuals. Beta-alanine-linked pyrrole-imidazole polyamides bind GAA.TTC tracts with high affinity and disrupt the intramolecular DNA.DNA-associated region of the sticky-DNA conformation formed by long GAA.TTC repeats. Fluorescent polyamide-Bodipy conjugates localize in the nucleus of a lymphoid cell line derived from a FRDA patient. The synthetic ligands increase transcription of the frataxin gene in cell culture, resulting in increased levels of frataxin protein. DNA microarray analyses indicate that a limited number of genes are significantly affected in FRDA cells. Polyamides may increase transcription by altering the DNA conformation of genes harboring long GAA.TTC repeats or by chromatin opening. | lld:pubmed |
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| pubmed-article:16857735 | pubmed:language | eng | lld:pubmed |
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| pubmed-article:16857735 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:16857735 | pubmed:month | Aug | lld:pubmed |
| pubmed-article:16857735 | pubmed:issn | 0027-8424 | lld:pubmed |
| pubmed-article:16857735 | pubmed:author | pubmed-author:MelanderChris... | lld:pubmed |
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| pubmed-article:16857735 | pubmed:author | pubmed-author:SonLeslie SLS | lld:pubmed |
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| pubmed-article:16857735 | pubmed:volume | 103 | lld:pubmed |
| pubmed-article:16857735 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:16857735 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:16857735 | pubmed:pagination | 11497-502 | lld:pubmed |
| pubmed-article:16857735 | pubmed:dateRevised | 2009-11-18 | lld:pubmed |
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| pubmed-article:16857735 | pubmed:year | 2006 | lld:pubmed |
| pubmed-article:16857735 | pubmed:articleTitle | DNA sequence-specific polyamides alleviate transcription inhibition associated with long GAA.TTC repeats in Friedreich's ataxia. | lld:pubmed |
| pubmed-article:16857735 | pubmed:affiliation | Department of Molecular Biology, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA. | lld:pubmed |
| pubmed-article:16857735 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:16857735 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| pubmed-article:16857735 | pubmed:publicationType | Research Support, N.I.H., Extramural | lld:pubmed |
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