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  1. Article

    Molecular Mechanisms and Therapeutics for the GAA·TTC Expansion Disease Friedreich Ataxia

    Friedreich ataxia (FRDA), the most common inherited ataxia, is caused by transcriptional silencing of the nuclear FXN gene, encoding the essential mitochondrial protein frataxin. Currently, there is no approved t...

    Joel M. Gottesfeld in Neurotherapeutics (2019)

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    Protocol

    Evaluation of Histone Deacetylase Inhibitors as Therapeutics for Neurodegenerative Diseases

    Various neurodegenerative diseases are associated with aberrant gene expression. We recently identified a novel class of pimelic o-aminobenzamide histone deacetylase (HDAC) inhibitors that show promise as therape...

    Elisabetta Soragni, Chun** Xu, Andrew Cooper, Heather L. Plasterer in Neurodegeneration (2011)

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    Article

    Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

    The trafficking defect associated with the cystic fibrosis disease-linked allele of the CFTR chloride channel is alleviated by inhibitors of histone deacetylases, suggesting that altering the transcription of ...

    Darren M Hutt, David Herman, Ana P C Rodrigues, Sabrina Noel in Nature Chemical Biology (2010)

  4. Article

    Correction: Corrigendum: Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia

    Nature Chemical Biology 2, 551–558 (2006); published online 20 August 2006; corrected after print 22 May 2007 In the version of this article initially published, no competing financial interests were declared....

    David Herman, Kai Jenssen, Ryan Burnett, Elisabetta Soragni in Nature Chemical Biology (2007)

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    Article

    Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia

    Expansion of GAA·TTC triplets within an intron in FXN (the gene encoding frataxin) leads to transcription silencing, forming the molecular basis for the neurodegenerative disease Friedreich's ataxia. Gene silenci...

    David Herman, Kai Jenssen, Ryan Burnett, Elisabetta Soragni in Nature Chemical Biology (2006)

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    Article

    Alkylation of duplex DNA in nucleosome core particles by duocarmycin SA and yatakemycin

    John D Trzupek, Joel M Gottesfeld, Dale L Boger in Nature Chemical Biology (2006)

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    Article

    Domain packing and dynamics in the DNA complex of the N-terminal zinc fingers of TFIIIA

    The three N-terminal zinc fingers of transcription factor IIIA bind in the DNA major groove. Substantial packing interfaces are formed between adjacent fingers, the linkers lose their intrinsic flexibility upo...

    Mark P. Foster, Deborah S. Wuttke, Ishwar Radhakrishnan in Nature Structural Biology (1997)

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    Article

    Regulation of gene expression by small molecules

    Small molecules that target specific DNA sequences have the potential to control gene expression. Ligands designed for therapeutic application must bind any predetermined DNA sequence with high affinity and pe...

    Joel M. Gottesfeld, Laura Neely, John W. Trauger, Eldon E. Baird, Peter B. Dervan in Nature (1997)

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    Article

    Transcription factor IIIA induced bending of the Xenopus somatic 5S gene promoter

    TRANSCRIPTION factor IIIA (TFIIIA), the canonical zinc-finger protein, is a protein of relative molecular mass 39,000 (39K) that is required for transcription of 5S-ribosomal subunit genes in Xenopus1. It binds i...

    Gary P. Schroth, Gary R. Cook, E. Morion Bradbury, Joel M. Gottesfeld in Nature (1989)

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    Article

    Eukaryotic transcription complexes

    Accurate transcription of eukaryotic genes in vitro is preceeded by the assembly of the template DNA into stable DNA-protein complexes. Such complexes have been reported for genes transcribed by each of the three...

    Cecilia H. von Beroldingen, Wanda F. Reynolds in Molecular and Cellular Biochemistry (1984)

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    Article

    Identifier sequences are transcribed specifically in brain

    ‘Identifier’ or ID sequences are present in 62% of the RNA polymerase II and III transcripts made in vitro from brain nuclei but in fewer than 4% of the transcripts made from the nuclei of other tissues. An homol...

    J. Gregor Sutcliffe, Robert J. Milner, Joel M. Gottesfeld, Richard A. Lerner in Nature (1984)

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    Chapter

    Chromatin Fractionation and the Properties of Transcriptionally Active Regions of Chromatin

    It is now well established that in the eukaryotic cell nucleus chromatin is organized into repeating subunits or nucleosomes composed of about 200 base pairs of double stranded DNA in association with two mole...

    Joel M. Gottesfeld in Chromatin Structure and Function (1979)