Results 81 to 90 of about 3,816 (187)
Progeria: Translational insights from cell biology [PDF]
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
core +1 more source
Clinicopathological Challenge: A Progressively Enlarging Hardened Skin Plaque
ABSTRACT Stiff skin syndrome (SSS) is a rare connective tissue disease manifesting as a progressive, non‐inflammatory fibrosis that causes the skin and soft tissues to harden. It can result in restricted joint movement, particularly affecting the shoulder and pelvic girdle. A segmental variant with a better prognosis has been described.
Marian Fernández Martínez +3 more
wiley +1 more source
Microvascular Health as a Key Determinant of Organismal Aging
Abstract Aging is a complex, multifaceted process affecting all organ systems, with vascular aging playing a central role in organismal health decline. Beyond its role in circulation, the vascular system acts as a dynamic interface between tissues, influencing countless physiological functions such as tissue regeneration and repair, immune responses ...
Mattia Cenciarini +4 more
wiley +1 more source
MG132 Induces Progerin Clearance and Improves Disease Phenotypes in HGPS-like Patients’ Cells
Progeroid syndromes (PS), including Hutchinson-Gilford Progeria Syndrome (HGPS), are premature and accelerated aging diseases, characterized by clinical features mimicking physiological aging.
Karim Harhouri +9 more
doaj +1 more source
Accumulation of prelamin A compromises NF-kB-regulated B-lymphopoiesis in progeria mouse model. [PDF]
published_or_final_versio
Liu, B +5 more
core +1 more source
Summary Hutchinson-Gilford progeria syndrome (HGPS, OMIM 176670) is a rare disorder characterized by segmental accelerated aging and early death from coronary artery disease or stroke. Nearly 90% of HGPS sufferers carry a G608G mutation within exon 11 of
Vera Wenzel +7 more
doaj +1 more source
iTRAQ-Based Analysis of Progerin Expression Reveals Mitochondrial Dysfunction, Reactive Oxygen Species Accumulation and Altered Proteostasis [PDF]
[Abstract] Introduction. Nuclear accumulation of a mutant form of the nuclear protein Lamin-A, called Progerin (PG) or Lamin AΔ50, occurs in Hutchinson-Gilford Progeria Syndrome (HGPS) or Progeria, an accelerated aging disease. One of the main symptoms
Arufe, M.C. +10 more
core +3 more sources
Endothelial progerin expression causes cardiovascular pathology through an impaired mechanoresponse
Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disorder characterized by accelerated cardiovascular disease with extensive fibrosis. It is caused by a mutation in LMNA leading to expression of truncated prelamin A (progerin) in the nucleus.
Osmanagic-Myers, Selma +10 more
openaire +3 more sources
Hutchinson–Gilford Progeria Syndrome (HGPS) is an ultra-rare human premature aging disorder that precipitates death because of cardiac disease. Almost all cases of HGPS are caused by aberrant splicing of the LMNA gene that results in the production of a ...
So-mi Kang +9 more
doaj +1 more source
Human longevity and common variations in the LMNA gene: a meta‐analysis [PDF]
Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/92016/1/j.1474-9726.2012.00808.x ...
Anselmi +42 more
core +1 more source

