نتایج جستجو برای: gilford progeria syndrome

تعداد نتایج: 622251  

Journal: :Biophysical Journal 2021

The nuclear morphology of eukaryotic cells is determined by the interplay between lamina forming skeleton, chromatin inside nucleus, and coupling with cytoskeleton. Nuclear alterations are often associated pathological conditions as in Hutchinson-Gilford progeria syndrome (HGPS), which a mutation lamin A gene yields an altered form protein, named progerin, aberrant shape. While genetic molecula...

2015
Jean-Ha Baek Eva Schmidt Nikenza Viceconte Charlotte Strandgren Karin Pernold Thibaud J. C. Richard Fred W. Van Leeuwen Nico P. Dantuma Peter Damberg Kjell Hultenby Brun Ulfhake Enrico Mugnaini Björn Rozell Maria Eriksson

Hutchinson-Gilford progeria syndrome (HGPS) is a segmental progeroid syndrome with multiple features suggestive of premature accelerated aging. Accumulation of progerin is thought to underlie the pathophysiology of HGPS. However, despite ubiquitous expression of lamin A in all differentiated cells, the HGPS mutation results in organ-specific defects. For example, bone and skin are strongly affe...

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