Results 71 to 80 of about 1,306,775 (184)

Craniofacial Abnormalities in Hutchinson-Gilford Progeria Syndrome [PDF]

open access: yesAmerican Journal of Neuroradiology, 2012
HGPS is a rare syndrome of segmental premature aging. Our goal was to expand the scope of structural bone and soft-tissue craniofacial abnormalities in HGPS through CT or MR imaging. Using The Progeria Research Foundation Medical and Research Database, 98 imaging studies on 25 patients, birth to 14.1 years of age, were comprehensively reviewed.
N J, Ullrich   +3 more
openaire   +2 more sources

Multiscale Architecture and Mechanics of the Cell Nucleus: Implications for Disease, Bioengineering and Nanomedicine

open access: yesAdvanced Science, Volume 13, Issue 37, 3 July 2026.
Nuclear mechanical properties are inherently scale‐dependent, arising from a hierarchical architecture that spans DNA, chromatin, the nuclear envelope, and condensates. Experimental techniques and theoretical models are integrated into a cohesive multiscale framework linking nanoscale structural features to organelle‐level mechanical behavior.
Xinran Liu   +15 more
wiley   +1 more source

The epidemiology of premature aging and associated comorbidities

open access: yesClinical Interventions in Aging, 2013
Fabio Coppedè Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy Abstract: Hutchinson–Gilford Progeria Syndrome and Werner syndrome, also known as childhood- and adulthood ...
Coppedè F
doaj  

Nuclear dysfunction in aging and neurodegeneration

open access: yesAlzheimer's &Dementia, Volume 22, Issue 7, July 2026.
Abstract Neurodegenerative diseases are characterized by a loss of neuronal function and structure, often in a region‐specific manner. Multiple factors contribute to neuronal dysfunction and death, including pathogenic protein buildup, protein mislocalization, and inflammation. Despite extensive research, the common mechanisms driving neurodegeneration
Abbigael Aday   +7 more
wiley   +1 more source

Cellular Senescence and Aging: Mechanisms, Disease Convergence, and Therapeutic Frontiers

open access: yesMedComm, Volume 7, Issue 7, July 2026.
This schematic illustrates the hierarchical and interconnected nature of the primary molecular hallmarks of aging. The progression of aging is driven by a convergence of intrinsic molecular insults. Within the nucleus, genomic instability and telomere attrition trigger persistent DDR, accompanied by extensive epigenetic alterations.
Guowei Cai   +10 more
wiley   +1 more source

A case of hutchinson-gilford progeria syndrome mimicking scleredema in early infancy

open access: yes, 1994
We report a case of Hutchinson-Gilford progeria syndrome (HGPS). The patient showed the characteristics of scleredema at the age of 2.5 months but developed all the manifestations of HGPS gradually until 10 months old.
Osma, E.   +7 more
core   +1 more source

Single Cell Mechanics in Disease Progression

open access: yesSmall Science, Volume 6, Issue 7, July 2026.
Cells transmit distinct mechanical forces through ECM adhesion and cell–cell junctions and actomyosin‐generated traction forces are transmitted to the substrate through integrin‐based focal adhesions. Mechanical signals are further transmitted to the nucleus via the LINC complex, connecting the cytoskeleton to the nuclear lamina. These forces integrate
Sabin Kim   +3 more
wiley   +1 more source

Hutchinson-Gilford syndrome

open access: yes, 2023
Hutchinsonův-Gilfordův syndrom patří do skupiny onemocnění nazývající se progerie. Jedná se o syndrom předčasného stárnutí, způsobený mutací genu pro lamin A, tzv. laminopatii.
Jurkovičová, Monika
core   +1 more source

Hutchinson-Gilford Progeria Syndrome

open access: yes, 2013
Hutchinson-Gilford Progeria Syndrome (HGPS) is a lethal congenital disorder, characterised by premature appearance of accelerated ageing in children. Although HGPS was first descri‐ bed by Jonathan Hutchinson [1] and then by Hastings Gilford [2] more than a century ago, it was not until 2003 that the genetic basis of HGPS was uncovered [3, 4 ...
Jean-Ha Baek   +2 more
openaire   +2 more sources

Karyopherin Dysfunction Is a Key Driver of Aging

open access: yesAging Cell, Volume 25, Issue 7, July 2026.
Studies on the proteostasis network and aging have focused on protein synthesis, folding, and turnover. Here, emphasis on the spatial dimension in proteostasis is proposed by recognizing nucleocytoplasmic transport as an important player in aging. ABSTRACT Aging is often framed as the gradual erosion of proteostasis, driven by declining chaperone ...
Louis R. Lapierre
wiley   +1 more source

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