نتایج جستجو برای: progeria

تعداد نتایج: 858  

Journal: :Experimental gerontology 2004
Joanna M Bridger Ian R Kill

Hutchinson-Gilford progeria syndrome is a rare genetic disorder that mimics certain aspects of aging prematurely. Recent work has revealed that mutations in the lamin A gene are a cause of the disease. We show here that cellular aging of Hutchinson-Gilford progeria syndrome fibroblasts is characterised by a period of hyperproliferation and terminates with a large increase in the rate of apoptos...

Journal: :Journal of cell science 2012
Chin Yee Ho Jan Lammerding

Lamins, the major components of the nuclear lamina, have gained rapidly increasing interest over the past decade as lamin mutations were found to cause numerous devastating diseases. These laminopathies include Emery–Dreifuss muscular dystrophy (EDMD), dilated cardiomyopathy type 1A, limb-girdle muscular dystrophy type 1B, familial partial lipodystrophy (FPLD), Charcot–Marie–Tooth disease type ...

Journal: :Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 2008
O P Sharma H Shigemitsu

In the years preceding and following the turn of the 19th century several publications appeared independently that drew attention to what is now regarded as sarcoidosis. The first recorded and illustrated example is attributed to Jonathan Hutchinson of London. It appeared in Illustrations of Clinical Surgery (1877).

Journal: :Archives of Disease in Childhood 1957

2011
Eduardo Mansilla Vanina Díaz Aquino Daniel Zambón Gustavo Horacio Marin Karina Mártire Gustavo Roque Thomas Ichim Neil H. Riordan Amit Patel Flavio Sturla Gustavo Larsen Rubén Spretz Luis Núñez Carlos Soratti Ricardo Ibar Michiel van Leeuwen José María Tau Hugo Drago Alberto Maceira

One of the most important and complex diseases of modern society is metabolic syndrome. This syndrome has not been completely understood, and therefore an effective treatment is not available yet. We propose a possible stem cell mechanism involved in the development of metabolic syndrome. This way of thinking lets us consider also other significant pathologies that could have similar etiopathog...

Journal: :Biophysical chemistry 2017
Gianmarco Ferri Barbara Storti Ranieri Bizzarri

Recent data indicate that nuclear lamina (NL) plays a relevant role in many fundamental cellular functions. The peculiar role of NL in cells is dramatically demonstrated by the Hutchinson-Gilford progeria syndrome (HGPS), an inherited laminopathy that causes premature, rapid aging shortly after birth. In HGPS, a mutant form of Lamin A (progeria) leads to a dysmorphic NL structure, but how this ...

2013
Oliver Dreesen Alexandre Chojnowski Peh Fern Ong Tian Yun Zhao John E. Common Declan Lunny E. Birgitte Lane Shu Jin Lee Leah A. Vardy Colin L. Stewart Alan Colman

The nuclear lamina consists of A- and B-type lamins. Mutations in LMNA cause many human diseases, including progeria, a premature aging syndrome, whereas LMNB1 duplication causes adult-onset autosomal dominant leukodystrophy (ADLD). LMNB1 is reduced in cells from progeria patients, but the significance of this reduction is unclear. In this paper, we show that LMNB1 protein levels decline in sen...

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