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Progeria: Translational insights from cell biology [PDF]
, 2012 Cell biologists love to think outside the box, pursuing many surprising twists and unexpected turns in their quest to unravel the mysteries of how cells work. But can cell biologists think outside the bench? We are certain that they can, and clearly some
Cao, Kan +2 more
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Oncology Reports, 2017 Ultraviolet (UV) radiation is the primary risk factor underlying photoaging and photocarcinogenesis. Mounting research has focused on the role of DNA damage response pathways in UV-induced double-strand break (DSB) repair. In the present study, we hypothesized that UVA-induced aberrant progerin upregulation may adversely affect p53-binding protein 1 ...
Xin, Huang +6 more
openaire +3 more sources
Mouse models for understanding the molecular mechanism of bone disease in Hutchinson-Gilford progeria syndrome [PDF]
, 2011 Aging is a complex process affecting all people. Intense research is applied to elucidate the biological basis of aging and disease that develop with aging.
Schmidt, Eva
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Research Models for Studying Vascular Calcification [PDF]
, 2020 Calcification of the vessel wall contributes to high cardiovascular morbidity and mortality. Vascular calcification (VC) is a systemic disease with multifaceted contributing and inhibiting factors in an actively regulated process.
Babic, Milen +4 more
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Accumulation of prelamin A compromises NF-kB-regulated B-lymphopoiesis in progeria mouse model. [PDF]
, 2013 published_or_final_versio
Liu, B +5 more
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Progerin, an Aberrant Spliced Form of Lamin A, Is a Potential Therapeutic Target for HGPS
Cells, 2023 Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare genetic disorder caused by the mutant protein progerin, which is expressed by the abnormal splicing of the LMNA gene.
Bae-Hoon Kim +5 more
doaj +1 more source
Scientific Reports, 2021 Children with Hutchinson–Gilford Progeria Syndrome (HGPS) suffer from multiple cardiovascular pathologies due to the expression of progerin, a mutant form of the nuclear envelope protein Lamin A.
Ryan von Kleeck +5 more
doaj +1 more source
Hutchinson Gilford Progeria Syndrome: A Therapeutic Approach via Adenoviral Delivery of CRISPR/cas Genome Editing System [PDF]
, 2014 Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare human genetic disease caused by mutations in the LMNA gene. LMNA codes for structural components of the nuclear lamina. Alterations of nuclear lamina lead to a very variable class of diseases known as
ARANCIO, Walter +3 more
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Role of progerin-induced telomere dysfunction in HGPS premature cellular senescence [PDF]
Journal of Cell Science, 2010 Hutchinson-Gilford Progeria Syndrome (HGPS) is a premature-aging syndrome caused by a dominant mutation in the gene encoding lamin A, which leads to an aberrantly spliced and processed protein termed progerin. Previous studies have shown that progerin induces early senescence associated with increased DNA-damage signaling and that telomerase extends ...
Erica K, Benson +2 more
openaire +2 more sources
JCI Insight, 2021 The mutant nuclear lamin protein (progerin) produced in Hutchinson-Gilford progeria syndrome (HGPS) results in loss of arterial smooth muscle cells (SMCs), but the mechanism has been unclear. We found that progerin induces repetitive nuclear membrane (NM)
Paul H. Kim +9 more
doaj +1 more source