Diane Merry

Summary

Affiliation: Thomas Jefferson University
Country: USA

Publications

  1. ncbi request reprint Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects
    Ruth Luthi-Carter
    Center for Aging, Genetics and Neurodegeneration, Massachusetts General Hospital, Charlestown, MA 02129 4404, USA
    Hum Mol Genet 11:1927-37. 2002
  2. ncbi request reprint Castration restores function and neurofilament alterations of aged symptomatic males in a transgenic mouse model of spinal and bulbar muscular atrophy
    Erica S Chevalier-Larsen
    Department of Biochemistry and Molecular Pharmacology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA
    J Neurosci 24:4778-86. 2004
  3. ncbi request reprint ASC-J9 ameliorates spinal and bulbar muscular atrophy phenotype via degradation of androgen receptor
    Zhiming Yang
    George Whipple Lab for Cancer Research, Departments of Pathology, Urology, and Radiation Oncology, and the Cancer Center, University of Rochester Medical Center, Rochester, New York 14642, USA
    Nat Med 13:348-53. 2007
  4. pmc Testosterone treatment fails to accelerate disease in a transgenic mouse model of spinal and bulbar muscular atrophy
    Erica S Chevalier-Larsen
    Department of Biochemistry and Molecular Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA
    Dis Model Mech 5:141-5. 2012
  5. pmc Animal models of Kennedy disease
    Diane E Merry
    Department of Biochemistry and Molecular Pharmacology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA
    NeuroRx 2:471-9. 2005
  6. pmc FOXO3a is broadly neuroprotective in vitro and in vivo against insults implicated in motor neuron diseases
    Jelena Mojsilovic-Petrovic
    Department of Pediatrics, Division of Neurology, Abramson Research Center, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Neurosci 29:8236-47. 2009
  7. ncbi request reprint Trinucleotide repeat disease. The androgen receptor in spinal and bulbar muscular atrophy
    Jessica L Walcott
    Department of Biochemistry and Molecular Pharmacology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA
    Vitam Horm 65:127-47. 2002
  8. pmc Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy
    Heather L Montie
    Department of Biochemistry and Molecular Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA
    Hum Mol Genet 18:1937-50. 2009
  9. ncbi request reprint Genetic modulation of polyglutamine toxicity by protein conjugation pathways in Drosophila
    H Y Edwin Chan
    Howard Hughes Medical Institute, Department of Biology, University of Pennsylvania, Philadelphia, PA 19104, USA
    Hum Mol Genet 11:2895-904. 2002
  10. ncbi request reprint Ligand promotes intranuclear inclusions in a novel cell model of spinal and bulbar muscular atrophy
    Jessica L Walcott
    Department of Biochemistry and Molecular Pharmacology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA
    J Biol Chem 277:50855-9. 2002

Research Grants

  1. POLYGLUTAMINE NEUROTOXICITY IN SBMA
    Diane E Merry; Fiscal Year: 2010
  2. Hormonal Control of SBMA Pathogenesis
    Diane Merry; Fiscal Year: 2007
  3. 2007 CAG Triplet Repeat Disorders Gordon Conference
    Diane Merry; Fiscal Year: 2007

Collaborators

  • Jon Clardy
  • Itzhak Mano
  • H Y Edwin Chan
  • J Paul Taylor
  • S Finkbeiner
  • Andrew Lieberman
  • Albert R La Spada
  • Christopher Ross
  • Andrew D Strand
  • Erica S Chevalier-Larsen
  • Heather L Montie
  • Jessica L Walcott
  • Yuhong Liu
  • Jelena Mojsilovic-Petrovic
  • Mei Li
  • Zhiming Yang
  • Latia Holder
  • Lisa M Mandrusiak
  • Christine K Bailey
  • Ruth Luthi-Carter
  • Robert G Kalb
  • Marco Boccitto
  • Weiguo Zhou
  • Monica Driscoll
  • Maria S Cho
  • Savvas N Georgiades
  • Andrey S Tsvetkov
  • Natalia Nedelsky
  • Rachael L Neve
  • Shu Shi Chang
  • Gen Sobue
  • I Chen Yu
  • Shuyuan Yeh
  • Marc I Diamond
  • Yu Jia Chang
  • Lu Min Chen
  • Chawnshang Chang
  • Cheng Chia Wu
  • Hiroshi Miyamoto
  • Huiyi Wang
  • Christopher J O'Brien
  • Shannon C Jenkins
  • Lenore K Beitel
  • Thomas C Scanlon
  • Erica Chevalier-Larsen
  • Xiaoling Wang
  • Mark A Trifiro
  • Gabriele Schilling
  • David R Borchelt
  • Sarah A Hanson
  • Anne B Young
  • Isabella F M Andriola
  • Charles Kooperberg
  • James M Olson
  • Harm H Kampinga

Detail Information

Publications14

  1. ncbi request reprint Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects
    Ruth Luthi-Carter
    Center for Aging, Genetics and Neurodegeneration, Massachusetts General Hospital, Charlestown, MA 02129 4404, USA
    Hum Mol Genet 11:1927-37. 2002
    ..These results demonstrate that some of the gene expression effects of expanded polyglutamine proteins occur independently of protein context...
  2. ncbi request reprint Castration restores function and neurofilament alterations of aged symptomatic males in a transgenic mouse model of spinal and bulbar muscular atrophy
    Erica S Chevalier-Larsen
    Department of Biochemistry and Molecular Pharmacology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA
    J Neurosci 24:4778-86. 2004
    ..These data suggest that hormone-based therapies designed to treat SBMA patients, even with advanced disease, are likely to be effective...
  3. ncbi request reprint ASC-J9 ameliorates spinal and bulbar muscular atrophy phenotype via degradation of androgen receptor
    Zhiming Yang
    George Whipple Lab for Cancer Research, Departments of Pathology, Urology, and Radiation Oncology, and the Cancer Center, University of Rochester Medical Center, Rochester, New York 14642, USA
    Nat Med 13:348-53. 2007
    ..Collectively, our results point to a better therapeutic and preventative approach to treating SBMA, by disrupting the interaction between AR and AR coregulators...
  4. pmc Testosterone treatment fails to accelerate disease in a transgenic mouse model of spinal and bulbar muscular atrophy
    Erica S Chevalier-Larsen
    Department of Biochemistry and Molecular Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA
    Dis Model Mech 5:141-5. 2012
    ....
  5. pmc Animal models of Kennedy disease
    Diane E Merry
    Department of Biochemistry and Molecular Pharmacology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA
    NeuroRx 2:471-9. 2005
    ..Whereas differences, primarily in neuropathological findings, exist in these models, these differences may be exploited to begin to elucidate the most relevant pathological features of KD...
  6. pmc FOXO3a is broadly neuroprotective in vitro and in vivo against insults implicated in motor neuron diseases
    Jelena Mojsilovic-Petrovic
    Department of Pediatrics, Division of Neurology, Abramson Research Center, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Neurosci 29:8236-47. 2009
    ..These results indicate that activation of the DAF-16/FOXO3a pathway, genetically or pharmacologically, confers protection against the known causes of motor neuron diseases...
  7. ncbi request reprint Trinucleotide repeat disease. The androgen receptor in spinal and bulbar muscular atrophy
    Jessica L Walcott
    Department of Biochemistry and Molecular Pharmacology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA
    Vitam Horm 65:127-47. 2002
    ..The recent development of useful animal and cell models of SBMA will lead to increased understanding of disease pathogenesis, as well as to the development of new and better therapeutic strategies...
  8. pmc Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy
    Heather L Montie
    Department of Biochemistry and Molecular Biology, Thomas Jefferson University, Philadelphia, PA 19107, USA
    Hum Mol Genet 18:1937-50. 2009
    ..They also highlight a mechanistic basis for the requirement for nuclear localization in SBMA neurotoxicity, namely the lack of mutant AR removal by the autophagic protein degradation pathway...
  9. ncbi request reprint Genetic modulation of polyglutamine toxicity by protein conjugation pathways in Drosophila
    H Y Edwin Chan
    Howard Hughes Medical Institute, Department of Biology, University of Pennsylvania, Philadelphia, PA 19104, USA
    Hum Mol Genet 11:2895-904. 2002
    ..These data suggest that post-translational protein modification, including the ubiquitin/proteasome and the SUMO-1 pathways, modulate poly(Q) pathogenesis...
  10. ncbi request reprint Ligand promotes intranuclear inclusions in a novel cell model of spinal and bulbar muscular atrophy
    Jessica L Walcott
    Department of Biochemistry and Molecular Pharmacology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA
    J Biol Chem 277:50855-9. 2002
    ..This model mimics the formation of ubiquitinated intranuclear inclusions containing the amino-terminal portion of AR observed in patient tissue and reveals a role for ligand in the pathogenesis of SBMA...
  11. ncbi request reprint Autophagy and access: understanding the role of androgen receptor subcellular localization in SBMA
    Heather L Montie
    Thomas Jefferson University, Department of Biochemistry and Molecular Biology, Philadelphia, PA, USA
    Autophagy 5:1194-7. 2009
    ..Our findings indicate that therapeutic intervention to induce autophagy represents a potential potent benefit for SBMA, and that it likely does so by protecting SBMA motor neurons independent of a direct effect on mutant AR...
  12. ncbi request reprint Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy
    Christine K Bailey
    Department of Biochemistry and Molecular Pharmacology, Thomas Jefferson University, 208 Bluemle Life Sciences Building, 233 S 10th Street, Philadelphia, PA 19107, USA
    Hum Mol Genet 11:515-23. 2002
    ..Molecular chaperone enhancement of protein degradation points to the modulation of molecular chaperones as a potential therapeutic target for polyglutamine diseases...
  13. ncbi request reprint Transglutaminase potentiates ligand-dependent proteasome dysfunction induced by polyglutamine-expanded androgen receptor
    Lisa M Mandrusiak
    Lady Davis Institute for Medical Research, Sir Mortimer B Davis Jewish General Hospital, Montreal, Quebec, H3T 1E2, Canada
    Hum Mol Genet 12:1497-506. 2003
    ..Because of the central role the ubiquitin-proteasome degradation system plays in fundamental cellular processes, any alteration in its function could cause cell death, ultimately contributing to SBMA pathogenesis...
  14. ncbi request reprint Soluble androgen receptor oligomers underlie pathology in a mouse model of spinobulbar muscular atrophy
    Mei Li
    Department of Neurology, Department of Cellular and Molecular Pharmacology, University of California, San Francisco UCSF, California 94143, USA
    J Biol Chem 282:3157-64. 2007
    ..This is the first demonstration of soluble AR oligomers in vivo and suggests that they underlie neurodegeneration in SBMA...

Research Grants3

  1. POLYGLUTAMINE NEUROTOXICITY IN SBMA
    Diane E Merry; Fiscal Year: 2010
    ..The research proposed here should lead to a better understanding of the specific pathogenic mechanisms responsible for SBMA;these studies should lead, as well, to a better understanding of these other protein accumulation diseases. ..
  2. Hormonal Control of SBMA Pathogenesis
    Diane Merry; Fiscal Year: 2007
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  3. 2007 CAG Triplet Repeat Disorders Gordon Conference
    Diane Merry; Fiscal Year: 2007
    ..All participants will be required to present posters. Priority will be given to women, minorities, and persons with disabilities when selecting participants. [unreadable] [unreadable] [unreadable]..