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Scholar Results 1 - 10 of about 101 related to Sagelius: Reversible phenotype in a mouse model of Hutchinson-Gilford progeria syndrome. (0.12 sec) 

Reversible phenotype in a mouse model of Hutchinson-Gilford progeria syndrome


H Sagelius, Y Rosengardten, E Schmidt, C … - British Medical Journal, 2008 - jmg.bmj.com
Hutchinson–Gilford progeria syndrome (HGPS) is a rare progeroid syndrome
caused by mutations in the LMNA gene. Currently there is no treatment available
for HGPS, but promising results from several studies using farnesyl ...
Cited by 7 - Related articles - All 4 versions

A farnesyltransferase inhibitor prevents both the onset and late progression of …

- pnas.org
BC Capell, M Olive, MR Erdos, K Cao, DA … - Proceedings of the National Academy of Sciences, 2008 - National Acad Sciences
Hutchinson-Gilford progeria syndrome (HGPS) is the most dramatic form of human
premature aging. Death occurs at a mean age of 13 years, usually from heart
attack or stroke. Almost all cases of HGPS are caused by a de novo point ...
Cited by 8 - Related articles - All 15 versions

Phenotype and course of Hutchinson-Gilford progeria syndrome

- angrylapdog.com
MA Merideth, LB Gordon, S Clauss, V Sachdev … - The New England journal of medicine, 2008 - content.nejm.org.p.angrylapdog.com
Results 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. ...
Cited by 32 - Related articles - Cached - BL Direct - All 7 versions

Epidermal expression of the truncated prelamin A causing Hutchinson-Gilford progeria …

- oxfordjournals.org
Y Wang, AA Panteleyev, DM Owens, K … - Human Molecular Genetics, 2008 - Oxford Univ Press
Hutchinson–Gilford progeria syndrome (HGPS) is an accelerated aging disorder
caused by point mutation in LMNA encoding A-type nuclear lamins. The mutations
in LMNA activate a cryptic splice donor site, resulting in expression of a ...
Cited by 4 - Related articles - All 6 versions

Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated


SH Yang, DA Andres, HP Spielmann, SG … - The Journal of Clinical Investigation, 2008 - pubmedcentral.nih.gov
Hutchinson-Gilford progeria syndrome (HGPS), a rare disease that results in what
appears to be premature aging, is caused by the production of a mutant form of
prelamin A known as progerin. Progerin retains a farnesyl lipid anchor at ...
Cited by 5 - Related articles - All 7 versions

New approaches to progeria


MW Kieran, L Gordon, M Kleinman - Pediatrics, 2007 - Am Acad Pediatrics
Progeria (Hutchinson-Gilford progeria syndrome) is a rare genetic disorder that
offers considerable insight into the biology of premature aging. This review
summarizes the clinical characteristics of this disease and the underlying ...
Cited by 10 - Related articles - BL Direct - All 3 versions

Targeted transgenic expression of the mutation causing Hutchinson-Gilford progeria …

- biologists.org
H Sagelius, Y Rosengardten, M Hanif, MR … - Journal of Cell Science, 2008 - jcs.biologists.org
Hutchinson-Gilford progeria syndrome (HGPS) is a rare human genetic disorder
characterized by striking progeroid features. Clinical findings in the skin
include scleroderma, alopecia and loss of subcutaneous fat. HGPS is usually ...
Cited by 11 - Related articles - BL Direct - All 5 versions

Ankyrin G overexpression in Hutchinson-Gilford progeria syndrome fibroblasts identified …


J Wang, JF Robinson, CH O'Neil, JY Edwards, … - Journal of Human Genetics, 2006 - Springer
Abstract Hutchinson-Gilford progeria syndrome (HGPS; MIM 176670) is a rare
disease characterized by accelerated aging. In this study, light and immuno-
fluorescence microscopy were used to assess morpho- logical changes, ...
Cited by 6 - Related articles - BL Direct - All 6 versions

Treatment with a farnesyltransferase inhibitor improves survival in mice with a Hutchinson …

- nih.gov
SH Yang, X Qiao, LG Fong, SG Young - BBA-Molecular and Cell Biology of Lipids, 2008 - Elsevier
Hutchinson–Gilford progeria syndrome (HGPS) is a progeroid syndrome
characterized by multiple aging-like disease phenotypes. We recently reported
that a protein farnesyltransferase inhibitor (FTI) improved several disease ...
Cited by 15 - Related articles - All 3 versions

Hutchinson-Gilford Progeria


KN Shah, HW Kaiser, J Hanfland - members.medscape.com
Hutchinson-Gilford progeria syndrome (HGPS) is an extremely rare hereditary
disease that affects the skin, musculoskeletal system, and vasculature. HGPS is
characterized by signs of premature aging. The term progeria is derived ...
Related articles - Cached - All 3 versions


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