L
Leslie B. Gordon
Researcher at Brown University
Publications - 68
Citations - 8047
Leslie B. Gordon is an academic researcher from Brown University. The author has contributed to research in topics: Progeria & Progerin. The author has an hindex of 32, co-authored 64 publications receiving 6908 citations. Previous affiliations of Leslie B. Gordon include National Institutes of Health & Progeria Research Foundation.
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Journal ArticleDOI
Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome
Maria Eriksson,W. Ted Brown,Leslie B. Gordon,Leslie B. Gordon,Michael W. Glynn,Joel Singer,Laura J. Scott,Michael R. Erdos,Christiane M. Robbins,Tracy Moses,Peter Berglund,Amalia Dutra,Evgenia Pak,Sandra G. Durkin,Antonei B. Csoka,Michael Boehnke,Thomas W. Glover,Francis S. Collins +17 more
TL;DR: Evidence of mutations in lamin A (LMNA) as the cause of Hutchinson–Gilford progeria syndrome is presented, and the discovery of the molecular basis of this disease may shed light on the general phenomenon of human ageing.
Journal ArticleDOI
Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome.
Robert D. Goldman,Dale K. Shumaker,Michael R. Erdos,Maria Eriksson,Anne E. Goldman,Leslie B. Gordon,Leslie B. Gordon,Yosef Gruenbaum,Satya Khuon,Melissa G. Mendez,Renee Varga,Francis S. Collins +11 more
TL;DR: It is shown by light and electron microscopy that HGPS is associated with significant changes in nuclear shape, including lobulation of the nuclear envelope, thickening of thenuclear lamina, loss of peripheral heterochromatin, and clustering of nuclear pores.
Journal ArticleDOI
Phenotype and Course of Hutchinson–Gilford Progeria Syndrome
Melissa A. Merideth,Leslie B. Gordon,Sarah Clauss,Vandana Sachdev,Ann C.M. Smith,Monique B. Perry,Carmen C. Brewer,Christopher K. Zalewski,H. Jeffrey Kim,Beth Solomon,Brian P. Brooks,Lynn H. Gerber,Maria L. Turner,Demetrio L. Domingo,Thomas C. Hart,Jennifer Graf,James C. Reynolds,Andrea L. Gropman,Jack A. Yanovski,Marie Gerhard-Herman,Francis S. Collins,Elizabeth G. Nabel,Richard O. Cannon,William A. Gahl,Wendy J. Introne +24 more
TL;DR: Clinical investigations confirmed sclerotic skin, joint contractures, bone abnormalities, alopecia, and growth impairment in all 15 patients; cardiovascular and central nervous system sequelae were also documented.
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Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome
Brian C. Capell,Michael R. Erdos,James P. Madigan,James J. Fiordalisi,Renee Varga,Karen N. Conneely,Leslie B. Gordon,Channing J. Der,Adrienne D. Cox,Francis S. Collins +9 more
TL;DR: The results suggest that treatment with FTIs represents a potential therapy for patients with HGPS, and hypothesized that retention of the farnesyl group causes progerin to become permanently anchored in the nuclear membrane, disrupting proper nuclear scaffolding and causing the characteristic nuclear blebbing seen in HGPS cells.
Journal ArticleDOI
Cardiovascular Pathology in Hutchinson-Gilford Progeria: Correlation With the Vascular Pathology of Aging
Michelle Olive,Ingrid A. Harten,Richard N. Mitchell,Jeanette Beers,Karima Djabali,Kan Cao,Michael R. Erdos,Cecilia D. Blair,Birgit Funke,Leslie B. Smoot,Marie Gerhard-Herman,Jason T. Machan,Robert Kutys,Renu Virmani,Francis S. Collins,Thomas N. Wight,Elizabeth G. Nabel,Leslie B. Gordon +17 more
TL;DR: Vascular progerin generation in young non-HGPS individuals, which significantly increases throughout life, strongly suggests that proger in has a role in cardiovascular aging of the general population.