Results 101 to 110 of about 7,521 (211)

Transformation Resistance in a Premature Aging Disorder Identifies a Tumor-Protective Function of BRD4

Cell Reports, 2014
Summary: Advanced age and DNA damage accumulation are prominent risk factors for cancer. The premature aging disorder Hutchinson-Gilford progeria syndrome (HGPS) provides a unique opportunity for studying the interplay between DNA damage and aging ...
Patricia Fernandez, Paola Scaffidi, Elke Markert, Ji-Hyeon Lee, Sushil Rane, Tom Misteli   +5 more
doaj   +1 more source

Hutchinson-Gilford Progeria Syndrome

, 2013
Hutchinson-Gilford Progeria Syndrome (HGPS) is a lethal congenital disorder, characterised by premature appearance of accelerated ageing in children. Although HGPS was first descri‐ bed by Jonathan Hutchinson [1] and then by Hastings Gilford [2] more than a century ago, it was not until 2003 that the genetic basis of HGPS was uncovered [3, 4 ...
Baek, Jean-Ha, McKenna, Tomás, Eriksson, Maria   +2 more
openaire   +3 more sources

Hutchinson-Gilford syndrome (progeria)

Indian Journal of Dermatology, 2009
Progeria is a rare, autosomal dominant, progeroid disorder. In the world literature less than 100 cases have been reported to date. We present this case because of its rarity.
Surjushe Amar, Thakre Minal, Vasani Resham, Saple Dattatray   +3 more
doaj  

Alterations to nuclear architecture and genome behavior in senescent cells. [PDF]

, 2007
The organization of the genome within interphase nuclei, and how it interacts with nuclear structures is important for the regulation of nuclear functions.
Abney J.R., Agrelo R., Barboro P., Becker P.B., Bemiller P.M., Benn P.A., Berezney R., Bowman P.D., Bridger J.M., Bridger J.M., C. S. CLEMENTS, Campisi J., Chaly N., Chang K.S., Cremer T., de Lange T., Dell'Orco R.T., Dijkwel P.A., Ferbeyre G., Filesi I., Fisher A.G., Fleischer S., Goldman R.D., Gruenbaum Y., Haque F., Hernandez-Verdun D., Holliday R., Hozak P., I. R. KILL, I. S. MEHTA, J. M. BRIDGER, Kill I.R., Kreth G., Lezhava T., Li Y., M. FIGGITT, Mallette F.A., Manuelidis L., Mazin A.L., Mitsui Y., Mukherjee A.B., Nickerson J., Okabe J., Padiath Q.S., Pawelec G., Petrova N.V., Schardin M., Schulz L., Smilenov L.B., Strouboulis J., Tanabe H., van Driel R., Wang G.S.   +52 more
core   +1 more source

Progeroid Syndrome and Mutation in LMNA Gene: Report of Two Cases from Iran [PDF]

Journal of Kerman University of Medical Sciences, 2005
Two Iranian cases with very rare progeroid syndrome are reported. The first is a 24-year-old girl who has been healthy till her 13th birthday. From that time she has been suffering from a progressive generalized and multi-systemic illness.
Y Shafeghati, N Levy, G.M Martin
doaj  

Concise Review: Plasma and Nuclear Membranes Convey Mechanical Information to Regulate Mesenchymal Stem Cell Lineage [PDF]

, 2016
Numerous factors including chemical, hormonal, spatial, and physical cues determine stem cell fate. While the regulation of stem cell differentiation by soluble factors is well-characterized, the role of mechanical force in the determination of lineage ...
Fuchs, Robyn K., Rubin, Janet, Thompson, William R., Uzer, Gunes   +3 more
core   +1 more source

Altered Nuclear Functions in Progeroid Syndromes: a Paradigm for Aging Research

The Scientific World Journal, 2009
Syndromes of accelerated aging could provide an entry point for identifying and dissecting the cellular pathways that are involved in the development of age-related pathologies in the general population.
Baomin Li, Sonali Jog, Jose Candelario, Sita Reddy, Lucio Comai   +4 more
doaj   +1 more source

Hutchinson-Gilford progeria syndrome with severe calcific aortic valve stenosis

Annals of Pediatric Cardiology, 2011
Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging syndrome that results from mutation in the Laminin A gene. This case report of a 12-year-old girl with HGPS is presented for the rarity of the syndrome and the classical clinical ...
Natesh B Hanumanthappa, Ganigara Madhusudan, Jayaranganath Mahimarangaiah, Cholenahally N Manjunath   +3 more
doaj   +1 more source

The truncated prelamin A in Hutchinson-Gilford progeria syndrome alters segregation of A-type and B-type lamin homopolymers. [PDF]

, 2006
Hutchinson-Gilford progeria syndrome (HGPS) is a dominant autosomal premature aging syndrome caused by the expression of a truncated prelamin A designated progerin.
Buendia, Brigitte, Coppey-Moisan, Maïté, Courvalin, Jean-Claude, Delbarre, Erwan, Gaillard, Claire, Tramier, Marc   +5 more
core   +1 more source

Extraskeletal Calcifications in Hutchinson-Gilford Progeria Syndrome

Bone, 2019
Children with Hutchinson-Gilford progeria syndrome (HGPS), a rare premature aging disease, exhibit extraskeletal calcifications detected by radiographic analysis and on physical examination. The aim of this study was to describe the natural history and pathophysiology of these abnormal calcifications in HGPS, and to determine whether medications and/or
C M, Gordon, R H, Cleveland, K, Baltrusaitis, J, Massaro, R B, D'Agostino, M G, Liang, B, Snyder, M, Walters, X, Li, D T, Braddock, M E, Kleinman, M W, Kieran, L B, Gordon   +12 more
openaire   +3 more sources