Leslie B Gordon

Summary

Affiliation: Brown University
Country: USA

Publications

  1. pmc Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome
    Brian C Capell
    Genome Technology Branch, National Human Genome Research Institute, National Institutes of Health, 50 South Drive, MSC 8004, Bethesda, MD 20892 8004, USA
    Proc Natl Acad Sci U S A 102:12879-84. 2005
  2. pmc Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody
    Dayle McClintock
    Department of Dermatology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA
    Proc Natl Acad Sci U S A 103:2154-9. 2006
  3. pmc Age-dependent loss of MMP-3 in Hutchinson-Gilford progeria syndrome
    Ingrid A Harten
    Hope Heart Matrix Biology Program, Benaroya Research Institute at Virginia Mason, Seattle, Washington, USA
    J Gerontol A Biol Sci Med Sci 66:1201-7. 2011
  4. ncbi request reprint Impact of farnesylation inhibitors on survival in hutchinson-gilford progeria syndrome
    Leslie B Gordon
    From the Department of Pediatrics, Hasbro Children s Hospital and Warren Alpert Medical School of Brown University, Providence, RI L B G Department of Anesthesia, Division of Critical Care Medicine, Boston Children s Hospital and Harvard Medical School, Boston, MA L B G, M E K Department of Mathematics and Statistics, Boston University, Harvard Clinical Research Institute, Boston, MA J M, R B D Center for Gerontology and Health Care Research, Brown University, Providence, RI S E C, J B Department of Genetics, New York State Institute for Basic Research, Staten Island, NY W T B Hematology Oncology, Boston Children s Hospital, Division of Pediatric Oncology, Dana Farber Cancer Institute and Harvard Medical School, Boston, MA M W K
    Circulation 130:27-34. 2014
  5. doi request reprint Progeria: a paradigm for translational medicine
    Leslie B Gordon
    Department of Anesthesia, Boston Children s Hospital and Harvard Medical School, Boston, MA 02115, USA Department of Pediatrics, Hasbro Children s Hospital and Warren Alpert Medical School of Brown University, Providence, RI 02912, USA Electronic address
    Cell 156:400-7. 2014
  6. pmc Clinical trial of a farnesyltransferase inhibitor in children with Hutchinson-Gilford progeria syndrome
    Leslie B Gordon
    Department of Anesthesia, Boston Children s Hospital and Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 109:16666-71. 2012
  7. ncbi request reprint Highlights of the 2007 Progeria Research Foundation scientific workshop: progress in translational science
    Leslie B Gordon
    Warren Alpert Medical School of Brown University, Providence, RI 02912, USA
    J Gerontol A Biol Sci Med Sci 63:777-87. 2008
  8. ncbi request reprint Disease progression in Hutchinson-Gilford progeria syndrome: impact on growth and development
    Leslie B Gordon
    Department of Pediatrics, Rhode Island Hospital, Providence, Rhode Island, USA
    Pediatrics 120:824-33. 2007
  9. doi request reprint A prospective study of radiographic manifestations in Hutchinson-Gilford progeria syndrome
    Robert H Cleveland
    Pediatric Radiology, Children s Hospital Boston, Harvard Medical School, Boston, MA 02115, USA
    Pediatr Radiol 42:1089-98. 2012
  10. pmc Mechanisms of premature vascular aging in children with Hutchinson-Gilford progeria syndrome
    Marie Gerhard-Herman
    Division of Cardiology, Brigham and Women s Hospital and Harvard Medical School, Boston, MA, USA
    Hypertension 59:92-7. 2012

Collaborators

  • Anita Giobbie-Hurder
  • Ara Nazarian
  • Antonei B Csoka
  • Bryan P Toole
  • Armin Schwartzman
  • Marie Gerhard-Herman
  • Maria Eriksson
  • Francis S Collins
  • RALPH B D'AGOSTINO
  • Carlos Lopez-Otin
  • Robert H Cleveland
  • Susanna Y Huh
  • M W Kieran
  • Robert D Goldman
  • Alice Lichtenstein
  • Dayle McClintock
  • Joan M Lemire
  • Jillian F Rork
  • Renee Varga
  • Nicole J Ullrich
  • Brian C Capell
  • Monica Kleinman
  • David T Miller
  • Donna Neuberg
  • Ingrid A Harten
  • Catherine M Gordon
  • Thomas N Wight
  • Melissa A Merideth
  • April Bingham
  • Elizabeth G Nabel
  • Karima Djabali
  • Michael R Erdos
  • Jennifer T Huang
  • Marilyn G Liang
  • Monica E Kleinman
  • V Michelle Silvera
  • Yoon Jae Cho
  • Jason T Machan
  • Frank G Rothman
  • Robert J Doiron
  • Marsha A Moses
  • Rima S Zahr
  • Nicolle Quinn
  • Adam S Curatolo
  • Brian D Snyder
  • Simeon Taylor
  • Emily Von Scheven
  • Nancy Sebring
  • Christopher T Parker
  • Brian P Brooks
  • Carmen C Brewer
  • Monique B Perry
  • Frederick T Murphy
  • Lynn H Gerber
  • Lawrence K Jung
  • Demetrio L Domingo
  • Gloria C Higgins
  • Thomas C Hart
  • Steven R Bauer
  • Carol A Wallace
  • Ann C M Smith
  • Donald P Goldsmith
  • Scott A Vogelgesang
  • Harry L Gewanter
  • Phillip Gorden
  • Alan N Baer
  • Danielle Mercatante Carrick
  • Frederick W Miller
  • Lisa G Rider
  • Rafael F Rivas-Chacon
  • John Miller
  • James C Reynolds
  • S Ray Mitchell
  • Donald A Person
  • Elizabeth Chalom Candell
  • Robert M Rennebohm
  • Laura James-Newton
  • C Michael Knee
  • E Arthur
  • Patience H White
  • Ildy M Katona
  • Balu H Athreya
  • Peter N Malleson
  • Karyl S Barron
  • Christopher Zalewski
  • H Jeffrey Kim
  • Jack A Yanovski
  • Ira N Targoff
  • John F Bohnsack
  • Richard O Cannon

Detail Information

Publications22

  1. pmc Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome
    Brian C Capell
    Genome Technology Branch, National Human Genome Research Institute, National Institutes of Health, 50 South Drive, MSC 8004, Bethesda, MD 20892 8004, USA
    Proc Natl Acad Sci U S A 102:12879-84. 2005
    ..Last, treatment of both early- and late-passage human HGPS fibroblasts with FTIs resulted in significant reductions in nuclear blebbing. Our results suggest that treatment with FTIs represents a potential therapy for patients with HGPS...
  2. pmc Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody
    Dayle McClintock
    Department of Dermatology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA
    Proc Natl Acad Sci U S A 103:2154-9. 2006
    ..This finding suggests that accumulation of progerin is directly involved in vascular disease in progeria...
  3. pmc Age-dependent loss of MMP-3 in Hutchinson-Gilford progeria syndrome
    Ingrid A Harten
    Hope Heart Matrix Biology Program, Benaroya Research Institute at Virginia Mason, Seattle, Washington, USA
    J Gerontol A Biol Sci Med Sci 66:1201-7. 2011
    ..This metalloproteinase has the potential to serve as a biomarker of therapeutic efficacy when assessing treatments for HGPS...
  4. ncbi request reprint Impact of farnesylation inhibitors on survival in hutchinson-gilford progeria syndrome
    Leslie B Gordon
    From the Department of Pediatrics, Hasbro Children s Hospital and Warren Alpert Medical School of Brown University, Providence, RI L B G Department of Anesthesia, Division of Critical Care Medicine, Boston Children s Hospital and Harvard Medical School, Boston, MA L B G, M E K Department of Mathematics and Statistics, Boston University, Harvard Clinical Research Institute, Boston, MA J M, R B D Center for Gerontology and Health Care Research, Brown University, Providence, RI S E C, J B Department of Genetics, New York State Institute for Basic Research, Staten Island, NY W T B Hematology Oncology, Boston Children s Hospital, Division of Pediatric Oncology, Dana Farber Cancer Institute and Harvard Medical School, Boston, MA M W K
    Circulation 130:27-34. 2014
    ..The key elements necessary for this analysis are a robust natural history of survival and comparison with a sufficiently large patient population that has been treated for a sufficient time period with disease-targeting medications...
  5. doi request reprint Progeria: a paradigm for translational medicine
    Leslie B Gordon
    Department of Anesthesia, Boston Children s Hospital and Harvard Medical School, Boston, MA 02115, USA Department of Pediatrics, Hasbro Children s Hospital and Warren Alpert Medical School of Brown University, Providence, RI 02912, USA Electronic address
    Cell 156:400-7. 2014
    ..The progress made on the premature aging disorder Progeria is a shining example of the impact that studies of rare diseases can have. ..
  6. pmc Clinical trial of a farnesyltransferase inhibitor in children with Hutchinson-Gilford progeria syndrome
    Leslie B Gordon
    Department of Anesthesia, Boston Children s Hospital and Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 109:16666-71. 2012
    ..Results from this clinical treatment trial for children with HGPS provide preliminary evidence that lonafarnib may improve vascular stiffness, bone structure, and audiological status...
  7. ncbi request reprint Highlights of the 2007 Progeria Research Foundation scientific workshop: progress in translational science
    Leslie B Gordon
    Warren Alpert Medical School of Brown University, Providence, RI 02912, USA
    J Gerontol A Biol Sci Med Sci 63:777-87. 2008
  8. ncbi request reprint Disease progression in Hutchinson-Gilford progeria syndrome: impact on growth and development
    Leslie B Gordon
    Department of Pediatrics, Rhode Island Hospital, Providence, Rhode Island, USA
    Pediatrics 120:824-33. 2007
    ..We sought to more clearly define the bone and weight abnormalities in patients with progeria as potential outcome parameters for prospective clinical trials...
  9. doi request reprint A prospective study of radiographic manifestations in Hutchinson-Gilford progeria syndrome
    Robert H Cleveland
    Pediatric Radiology, Children s Hospital Boston, Harvard Medical School, Boston, MA 02115, USA
    Pediatr Radiol 42:1089-98. 2012
    ..There has been no comprehensive prospective study describing the skeletal abnormalities associated with progeria...
  10. pmc Mechanisms of premature vascular aging in children with Hutchinson-Gilford progeria syndrome
    Marie Gerhard-Herman
    Division of Cardiology, Brigham and Women s Hospital and Harvard Medical School, Boston, MA, USA
    Hypertension 59:92-7. 2012
    ....
  11. pmc Neurologic features of Hutchinson-Gilford progeria syndrome after lonafarnib treatment
    Nicole J Ullrich
    Department of Neurology, Boston Children s Hospital and Harvard Medical School, Boston, MA, USA
    Neurology 81:427-30. 2013
    ....
  12. doi request reprint Hutchinson-Gilford progeria is a skeletal dysplasia
    Catherine M Gordon
    Division of Adolescent Medicine and Endocrinology, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA
    J Bone Miner Res 26:1670-9. 2011
    ..Dietary intake was adequate, confirming that HGPS does not represent a model of malnutrition-induced bone loss. Taken together, these findings suggest that the phenotype of HGPS represents a unique skeletal dysplasia...
  13. ncbi request reprint Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome
    Maria Eriksson
    Department of Human Genetics, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, New York 10314, USA
    Nature 423:293-8. 2003
    ..The discovery of the molecular basis of this disease may shed light on the general phenomenon of human ageing...
  14. ncbi request reprint Aggrecan expression is substantially and abnormally upregulated in Hutchinson-Gilford Progeria Syndrome dermal fibroblasts
    Joan M Lemire
    Department of Anatomy and Cellular Biology, Tufts University School of Medicine, Boston, MA, USA
    Mech Ageing Dev 127:660-9. 2006
    ..This demonstrates that elevated aggrecan expression and its secretion are aberrant features of HGPS. We conclude that HGPS cells can display massively altered transcript levels leading to the secretion of inappropriate protein species...
  15. doi request reprint Initial cutaneous manifestations of Hutchinson-Gilford progeria syndrome
    Jillian F Rork
    Department of Dermatology, Boston Children s Hospital, Harvard Medical School, Boston, Massachusetts
    Pediatr Dermatol 31:196-202. 2014
    ..HGPS has distinct cutaneous manifestations during the first 2 years of life that may be the first signs of disease. Awareness of these findings could expedite diagnosis...
  16. ncbi request reprint Reduced adiponectin and HDL cholesterol without elevated C-reactive protein: clues to the biology of premature atherosclerosis in Hutchinson-Gilford Progeria Syndrome
    Leslie B Gordon
    Department of Anatomy and Cellular Biology, Tufts University School of Medicine, Boston, MA 02111, USA
    J Pediatr 146:336-41. 2005
    ..We determined whether children with HGPS demonstrate abnormalities in known biomarkers for cardiovascular disease (CVD) risk...
  17. ncbi request reprint Hyaluronan is not elevated in urine or serum in Hutchinson-Gilford Progeria Syndrome
    Leslie B Gordon
    Department of Anatomy and Cellular Biology, Tufts University School of Medicine, 136 Harrison Avenue, Boston, MA 02111, USA
    Hum Genet 113:178-87. 2003
    ..These studies indicate that neither serum nor urinary hyaluronan concentration is a reliable diagnostic or prognostic marker for HGPS and underscore a difference between adult and childhood progerias...
  18. pmc Progressive vascular smooth muscle cell defects in a mouse model of Hutchinson-Gilford progeria syndrome
    Renee Varga
    Genome Technology Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 103:3250-5. 2006
    ..This mouse model should prove valuable for testing experimental therapies for this devastating disorder and for exploring cardiovascular disease in general...
  19. pmc Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome
    Robert D Goldman
    Department of Cell and Molecular Biology, Northwestern University Feinberg School of Medicine, 303 East Chicago Avenue, Chicago, IL 60611, USA
    Proc Natl Acad Sci U S A 101:8963-8. 2004
    ....
  20. pmc Phenotype and course of Hutchinson-Gilford progeria syndrome
    Melissa A Merideth
    National Human Genome Research Institute, Intramural Office of Rare Disease, National Institutes of Health, Bethesda, MD 20892 1851, USA
    N Engl J Med 358:592-604. 2008
    ....
  21. pmc The mutant form of lamin A that causes Hutchinson-Gilford progeria is a biomarker of cellular aging in human skin
    Dayle McClintock
    Department of Dermatology, College of Physicians and Surgeons, Columbia University, New York, New York, United States of America
    PLoS ONE 2:e1269. 2007
    ..Our findings demonstrate that progerin expression is a biomarker of normal cellular aging and may potentially be linked to terminal differentiation and senescence in elderly individuals...
  22. pmc Predictors of acquired lipodystrophy in juvenile-onset dermatomyositis and a gradient of severity
    April Bingham
    From Office of Clinical Research, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, Maryland 20892 1301, USA
    Medicine (Baltimore) 87:70-86. 2008
    ..Further study is warranted to investigate the pathogenesis of acquired LD in patients with DM...