Robert Wilson

Summary

Affiliation: University of Pennsylvania
Country: USA

Publications

  1. ncbi request reprint Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue
    R B Wilson
    Department of Pathology, University of Pennsylvania, Philadelphia 19104, USA
    Nat Genet 16:352-7. 1997
  2. pmc Introduction of mismatches in a random shRNA-encoding library improves potency for phenotypic selection
    Yongping Wang
    Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America Department of Pathology and Laboratory Medicine, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
    PLoS ONE 9:e87390. 2014
  3. pmc A random shRNA-encoding library for phenotypic selection and hit-optimization
    Yongping Wang
    Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
    PLoS ONE 3:e3171. 2008
  4. pmc Therapeutic developments in Friedreich ataxia
    Robert B Wilson
    Department of Pathology and Laboratory Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA
    J Child Neurol 27:1212-6. 2012
  5. ncbi request reprint Iron dysregulation in Friedreich ataxia
    Robert B Wilson
    Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA 19106, USA
    Semin Pediatr Neurol 13:166-75. 2006
  6. ncbi request reprint Frataxin and frataxin deficiency in Friedreich's ataxia
    Robert B Wilson
    Department of Pathology and Laboratory Medicine, University of Pennsylvania, Room 509A, Stellar Chance Laboratories, 422 Curie Boulevard, Philadelphia 19104, USA
    J Neurol Sci 207:103-5. 2003
  7. ncbi request reprint Ferritin crystal cataracts in hereditary hyperferritinemia cataract syndrome
    David G Brooks
    Division of Medical Genetics, Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Invest Ophthalmol Vis Sci 43:1121-6. 2002
  8. ncbi request reprint Friedreich ataxia: effects of genetic understanding on clinical evaluation and therapy
    David R Lynch
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, USA
    Arch Neurol 59:743-7. 2002
  9. ncbi request reprint Near infrared muscle spectroscopy in patients with Friedreich's ataxia
    David R Lynch
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Muscle Nerve 25:664-73. 2002
  10. pmc Measuring the rate of progression in Friedreich ataxia: implications for clinical trial design
    Lisa S Friedman
    Department of Neurology, School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Mov Disord 25:426-32. 2010

Research Grants

  1. FUNCTIONAL STUDIES OF THE FRIEDREICHS ATAXIA PROTEIN
    Robert Wilson; Fiscal Year: 2002
  2. Identification of Anticancer Drug Targets
    Robert Wilson; Fiscal Year: 2002
  3. Random shRNA Selection
    Robert Wilson; Fiscal Year: 2009
  4. 3rd International Friedreich's Ataxia Scientific Conference
    Robert Wilson; Fiscal Year: 2007
  5. High throughput screen for cyclin D1 inhibitors (RMI)
    Robert Wilson; Fiscal Year: 2004
  6. Identification of Anticancer Drug Targets
    Robert Wilson; Fiscal Year: 2004
  7. Friedreich Ataxia High Throughput Drug Screening Assays
    Robert Wilson; Fiscal Year: 2004
  8. Identification of Anticancer Drug Targets
    Robert Wilson; Fiscal Year: 2003
  9. Friedreich's Ataxia Research Conference
    Robert Wilson; Fiscal Year: 2003
  10. Random shRNA Selection
    Robert B Wilson; Fiscal Year: 2010

Collaborators

Detail Information

Publications24

  1. ncbi request reprint Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue
    R B Wilson
    Department of Pathology, University of Pennsylvania, Philadelphia 19104, USA
    Nat Genet 16:352-7. 1997
    ..Our data suggest that mitochondrial dysfunction contributes to FRDA pathophysiology...
  2. pmc Introduction of mismatches in a random shRNA-encoding library improves potency for phenotypic selection
    Yongping Wang
    Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America Department of Pathology and Laboratory Medicine, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
    PLoS ONE 9:e87390. 2014
    ..The improved potency of our second-generation library expands the range of possible unbiased screens for small-RNA therapeutics and biologic tools. ..
  3. pmc A random shRNA-encoding library for phenotypic selection and hit-optimization
    Yongping Wang
    Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
    PLoS ONE 3:e3171. 2008
    ..Our library allows unbiased selection and optimization of shRNA-encoding sequences that confer phenotypes of interest, and could be used for the development of therapeutics and tools in many fields of biology...
  4. pmc Therapeutic developments in Friedreich ataxia
    Robert B Wilson
    Department of Pathology and Laboratory Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA
    J Child Neurol 27:1212-6. 2012
    ..These initiatives are in preclinical and clinical development and are reviewed herein...
  5. ncbi request reprint Iron dysregulation in Friedreich ataxia
    Robert B Wilson
    Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA 19106, USA
    Semin Pediatr Neurol 13:166-75. 2006
    ..Iron chelators and compounds that increase frataxin expression are under evaluation. Further elucidation of frataxin's function should lead to additional therapeutic approaches...
  6. ncbi request reprint Frataxin and frataxin deficiency in Friedreich's ataxia
    Robert B Wilson
    Department of Pathology and Laboratory Medicine, University of Pennsylvania, Room 509A, Stellar Chance Laboratories, 422 Curie Boulevard, Philadelphia 19104, USA
    J Neurol Sci 207:103-5. 2003
  7. ncbi request reprint Ferritin crystal cataracts in hereditary hyperferritinemia cataract syndrome
    David G Brooks
    Division of Medical Genetics, Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Invest Ophthalmol Vis Sci 43:1121-6. 2002
    ..HHCS was diagnosed in this study in one of the first families known to be affected in the United States, and the basis of lens opacities in HHCS was determined...
  8. ncbi request reprint Friedreich ataxia: effects of genetic understanding on clinical evaluation and therapy
    David R Lynch
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, USA
    Arch Neurol 59:743-7. 2002
    ..Although no proven therapy is yet available, antioxidants are a potential method for therapeutic intervention...
  9. ncbi request reprint Near infrared muscle spectroscopy in patients with Friedreich's ataxia
    David R Lynch
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Muscle Nerve 25:664-73. 2002
    ..These results suggest that NIRS may be an effective tool for monitoring the biochemical and functional features of Friedreich's ataxia in parallel...
  10. pmc Measuring the rate of progression in Friedreich ataxia: implications for clinical trial design
    Lisa S Friedman
    Department of Neurology, School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Mov Disord 25:426-32. 2010
    ..These results help to establish norms for progression in FRDA that can be useful in measuring the long-term success of therapeutic agents and defining sample-size calculations for double-blind clinical trials...
  11. ncbi request reprint Increased IRP1 activity in Friedreich ataxia
    Lioba Lobmayr
    University of Pennsylvania School of Medicine, Department of Pathology and Laboratory Medicine, Room 509A, Stellar Chance Laboratories, 422 Curie Blvd, Philadelphia, PA 19104, USA
    Gene 354:157-61. 2005
    ..Our data suggest an impaired adaptive response to iron accumulation in FRDA cells...
  12. ncbi request reprint Novel, complex interruptions of the GAA repeat in small, expanded alleles of two affected siblings with late-onset Friedreich ataxia
    Catherine A Stolle
    The Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104 4318, USA
    Mov Disord 23:1303-6. 2008
    ..Our observations suggest that interrupted GAA repeats do not clearly impact the age of onset in FA...
  13. ncbi request reprint Risk calculations for cystic fibrosis in neonatal screening by immunoreactive trypsinogen and CFTR mutation tests
    Shuji Ogino
    Department of Pathology, Brigham and Womens Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    Genet Med 7:317-27. 2005
    ..Although neonatal screening (or newborn screening) for cystic fibrosis (CF) is commonly practiced, systematic methods for accurate risk calculations are currently lacking...
  14. doi request reprint Pharmacotherapy for Friedreich ataxia
    Amy Y Tsou
    Department of Neurology, University of Pennsylvania School of Medicine, and Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104 4318, USA
    CNS Drugs 23:213-23. 2009
    ..However, continued development of new therapies will require creation of new, more sensitive measures for neurological dysfunction in FA, and clinically relevant measures of cardiac dysfunction...
  15. doi request reprint Urinary isoprostanes in Friedreich ataxia: lack of correlation with disease features
    Lauren M Myers
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
    Mov Disord 23:1920-2. 2008
    ..Thus, urinary F(2)-isoprostanes are not a useful biomarker in FA...
  16. ncbi request reprint 2003 International Friedreich's Ataxia Research Conference, 14-16 February 2003, Bethesda, MD, USA
    Hervé Seznec
    Institut de Genetique et de Biologie Moleculaire et Cellulaire IGBMC, CNRS INSERM Universite Louis Pasteur, 1 rue Laurent Fries BP 10142, Illkirch Cedex 67404, CU de Strasbourg, France
    Neuromuscul Disord 14:70-82. 2004
  17. pmc Spinal muscular atrophy genetic testing experience at an academic medical center
    Shuji Ogino
    Department of Pathology and Laboratory Medicine, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania 19104, USA
    J Mol Diagn 4:53-8. 2002
    ..We conclude that comprehensive SMA testing, including SMN1 deletion analysis, SMN1 gene dosage analysis, and linkage analysis, offers the most complete evaluation of SMA patients and their families...
  18. ncbi request reprint Transferrin receptor hyperexpression in primary erythroblasts is lost on transformation by avian erythroblastosis virus
    Lioba Lobmayr
    Institute of Medical Biochemistry, Division of Molecular Biology, Vienna Biocenter, Vienna, Austria
    Blood 100:289-98. 2002
    ..In summary, stabilization of TfR mRNA and redistribution of Fe-Tf/TfR complexes to late endosomal compartments may contribute to TfR hyperexpression in primary erythroblasts, effects that are lost on leukemic transformation...
  19. pmc Mortality in Friedreich's Ataxia
    David R Lynch
    Tex Heart Inst J 34:502-3; author reply 503-4. 2007
  20. pmc Alpha-synuclein-induced aggregation of cytoplasmic vesicles in Saccharomyces cerevisiae
    James H Soper
    Center for Neurodegenerative Disease Research and Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
    Mol Biol Cell 19:1093-103. 2008
    ....
  21. pmc Quantification of PCR bias caused by a single nucleotide polymorphism in SMN gene dosage analysis
    Shuji Ogino
    Department of Pathology, Brigham and Women s Hospital, Boston, MA 02115, USA
    J Mol Diagn 4:185-90. 2002
    ..As additional clinically significant single nucleotide polymorphisms (SNPs) are discovered, assessment of PCR bias, and judicious selection of standards and controls, will be increasingly important for quantitative PCR assays...
  22. pmc Advancements in the pathophysiology of Friedreich's Ataxia and new prospects for treatments
    Ngolela E Babady
    Department of Pediatric and Adolescent Medicine, Mayo Clinic College of Medicine, Rochester, MN 55905, USA, and Bruce Lefroy Centre for Genetic Health Research, Murdoch Children s Research Institute, Royal Children s Hospital, Melbourne, Australia
    Mol Genet Metab 92:23-35. 2007
    ..Story Landis noted in her opening remarks for the conference that there was a "palpable sense of energy, excitement, and enthusiasm" over the scientific progress made since the FRDA gene was discovered over 10 years ago...
  23. ncbi request reprint Comment on SMN2 deletion in childhood-onset spinal muscular atrophy
    Shuji Ogino
    Am J Med Genet 109:243-4; author reply 245. 2002
  24. pmc Antioxidant use in Friedreich ataxia
    Lauren Myers
    Department of Neurology, University of Pennsylvania School of Medicine, USA
    J Neurol Sci 267:174-6. 2008
    ..This confirms that non-prescription antioxidant use represents a major confounder to formal trials of existing and novel agents for Friedreich ataxia...

Research Grants12

  1. FUNCTIONAL STUDIES OF THE FRIEDREICHS ATAXIA PROTEIN
    Robert Wilson; Fiscal Year: 2002
    ..The results of these studies will further the understanding of the pathophysiology of Friedreich's Ataxia and are therefore relevant to the development of novel methods of prevention and treatment. ..
  2. Identification of Anticancer Drug Targets
    Robert Wilson; Fiscal Year: 2002
    ..The human homologs of genes identified in Aim 1 will be cloned, and the consequences of their mutation in appropriate cell lines will be determined. ..
  3. Random shRNA Selection
    Robert Wilson; Fiscal Year: 2009
    ..This approach has implications for the development of stem-cell-based and infectious-disease therapeutics, and is highly relevant to public health. ..
  4. 3rd International Friedreich's Ataxia Scientific Conference
    Robert Wilson; Fiscal Year: 2007
    ..These advances in therapeutic approaches will have direct applications to individuals with FRDA as well as to individuals with other rare and common diseases. ..
  5. High throughput screen for cyclin D1 inhibitors (RMI)
    Robert Wilson; Fiscal Year: 2004
    ....
  6. Identification of Anticancer Drug Targets
    Robert Wilson; Fiscal Year: 2004
    ..The human homologs of genes identified in Aim 1 will be cloned, and the consequences of their mutation in appropriate cell lines will be determined. ..
  7. Friedreich Ataxia High Throughput Drug Screening Assays
    Robert Wilson; Fiscal Year: 2004
    ..We will use a colony-color screening technique in the yeast model system to identify the target proteins of hit compounds from our high-throughput drug screening assays for FRDA. ..
  8. Identification of Anticancer Drug Targets
    Robert Wilson; Fiscal Year: 2003
    ..The human homologs of genes identified in Aim 1 will be cloned, and the consequences of their mutation in appropriate cell lines will be determined. ..
  9. Friedreich's Ataxia Research Conference
    Robert Wilson; Fiscal Year: 2003
    ....
  10. Random shRNA Selection
    Robert B Wilson; Fiscal Year: 2010
    ..This approach has implications for the development of stem-cell-based and infectious-disease therapeutics, and is highly relevant to public health. ..