نتایج جستجو برای: progeroid syndromes

تعداد نتایج: 81654  

2014
Ken Coates

No question is more important in Britain today than that of industrial democracy. More and more people ai'e keenly discussing the issue which quite recently was of concern only to the smallest minority. In the next year or so, it is perfectly possible that general elections will be fought in order to decide upon proposals for the democratisation of work. With the British economy stumbling from ...

Journal: :Frontiers in Neurology 2018

2008
A. ZVAVITCH

Abstract. The aim of this paper is to study properties of sections of convex bodies with respect to different types of measures. We present a formula connecting the Minkowski functional of a convex symmetric body K with the measure of its sections. We apply this formula to study properties of general measures most of which were known before only in the case of the standard Lebesgue measure. We ...

2014
Tomás McKenna Ylva Rosengardten Nikenza Viceconte Jean-Ha Baek Diana Grochová Maria Eriksson

Hutchinson-Gilford progeria syndrome (HGPS) and restrictive dermopathy (RD) are two laminopathies caused by mutations leading to cellular accumulation of prelamin A or one of its truncated forms, progerin. One proposed mechanism for the more severe symptoms in patients with RD compared with HGPS is that higher levels of farnesylated lamin A are produced in RD. Here, we show evidence in support ...

Journal: :FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2008
Yiyong Liu Youjie Wang Antonio E Rusinol Michael S Sinensky Ji Liu Steven M Shell Yue Zou

Cellular accumulation of DNA damage has been widely implicated in cellular senescence, aging, and premature aging. In Hutchinson-Gilford progeria syndrome (HGPS) and restrictive dermopathy (RD), premature aging is linked to accumulation of DNA double-strand breaks (DSBs), which results in genome instability. However, how DSBs accumulate in cells despite the presence of intact DNA repair protein...

Journal: :international journal of pediatrics 0
imran gattoo government medical college srinagar,india sudesh singh government medical college jammu ,india nucksheeba aziz government medical college srinagar,india

a female one month old with features supporting a diagnosis of neonatal progeroid syndrome(wrs)  presented to our neonatology section of gb pant children hospital srinagar .she had prenatal and post natal growth failure, generalized lipotrophy, triangular face, psedohydrocephalous, sparse scalp hair and eye brows, prominent scalp veins and greatly widened anterior fontenella.

Journal: :Cell reports 2013
Darren J Baker Robbyn L Weaver Jan M van Deursen

BubR1 insufficiency occurs with natural aging and induces progeroid phenotypes in both mice and children with mosaic variegated aneuploidy syndrome. In response to BubR1 insufficiency, skeletal muscle, fat, and lens tissue engage p19(Arf) to attenuate senescence and age-related deterioration. Here, we address how p19(Arf) exerts this caretaker role using BubR1 progeroid mice lacking p53 or its ...

2012
Mitra Lavasani Andria R. Robinson Aiping Lu Minjung Song Joseph M. Feduska Bahar Ahani Jeremy S. Tilstra Chelsea H. Feldman Paul D. Robbins Laura J. Niedernhofer Johnny Huard

With ageing, there is a loss of adult stem cell function. However, there is no direct evidence that this has a causal role in ageing-related decline. We tested this using muscle-derived stem/progenitor cells (MDSPCs) in a murine progeria model. Here we show that MDSPCs from old and progeroid mice are defective in proliferation and multilineage differentiation. Intraperitoneal administration of ...

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