Leslie B Gordon
Affiliation: Brown University
- Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibodyDayle 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...
- Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndromeBrian 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...
- Age-dependent loss of MMP-3 in Hutchinson-Gilford progeria syndromeIngrid 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...
- Clinical trial of a farnesyltransferase inhibitor in children with Hutchinson-Gilford progeria syndromeLeslie 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...
- Highlights of the 2007 Progeria Research Foundation scientific workshop: progress in translational scienceLeslie B Gordon
Warren Alpert Medical School of Brown University, Providence, RI 02912, USA
J Gerontol A Biol Sci Med Sci 63:777-87. 2008
- Disease progression in Hutchinson-Gilford progeria syndrome: impact on growth and developmentLeslie 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...
- A prospective study of radiographic manifestations in Hutchinson-Gilford progeria syndromeRobert 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...
- Mechanisms of premature vascular aging in children with Hutchinson-Gilford progeria syndromeMarie Gerhard-Herman
Division of Cardiology, Brigham and Women s Hospital and Harvard Medical School, Boston, MA, USA
Hypertension 59:92-7. 2012....
- Hutchinson-Gilford progeria is a skeletal dysplasiaCatherine 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...
- Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndromeMaria 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...
- Progressive vascular smooth muscle cell defects in a mouse model of Hutchinson-Gilford progeria syndromeRenee 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...
- Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndromeRobert 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....
- Aggrecan expression is substantially and abnormally upregulated in Hutchinson-Gilford Progeria Syndrome dermal fibroblastsJoan 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...
- Phenotype and course of Hutchinson-Gilford progeria syndromeMelissa 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....
- Predictors of acquired lipodystrophy in juvenile-onset dermatomyositis and a gradient of severityApril 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...
- Reduced adiponectin and HDL cholesterol without elevated C-reactive protein: clues to the biology of premature atherosclerosis in Hutchinson-Gilford Progeria SyndromeLeslie B Gordon
Department of Anatomy and Cellular Biology, Tufts University School of Medicine, Boston, MA 02111, USA
J Pediatr 146:336-41. 2005..HDL and adiponectin may represent significant mediators and potential therapeutic targets for atherosclerosis in HGPS...
- The mutant form of lamin A that causes Hutchinson-Gilford progeria is a biomarker of cellular aging in human skinDayle 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...