New look at the role of progerin in skin aging [PDF]
Current literature data indicate that progerin, which is a mutant of lamin A, may be one of several previously known physiological biomarkers of the aging process which begins at the age of 30. Lamins belong to the family of intermediate filaments type V
Anna Skoczyńska +3 more
doaj +3 more sources
Rare progerin-expressing preadipocytes and adipocytes contribute to tissue depletion over time [PDF]
Accumulation of progerin is believed to underlie the pathophysiology of Hutchinson-Gilford progeria syndrome, a disease characterized by clinical features suggestive of premature aging, including loss of subcutaneous white adipose tissue (sWAT). Although
Gwladys Revêchon +9 more
doaj +3 more sources
Prematurely Aged Human Microglia Exhibit Impaired Stress Response and Defective Nucleocytoplasmic Shuttling of ALS Associated FUS. [PDF]
We developed a human microglia aging model by doxycycline‐induced GFP‐progerin expression, which offers a valuable tool to study human microglial aging and its contribution to neurodegeneration. Induced HMC3‐Progerin cells exhibited increased age markers as well as transcriptomic alterations, including an age‐associated transcriptomic shift and ...
Hartmann C +16 more
europepmc +2 more sources
miR-140-5p Overexpression Contributes to Oxidative Stress and Mitochondrial Dysfunction in Hutchinson-Gilford Progeria Syndrome Fibroblasts Through NRF2 Pathway. [PDF]
This study identifies a novel molecular mechanism involving miR‐140‐5p that contributes to the pathogenesis of HGPS. By decreasing NRF2 expression, miR‐140‐5p overexpression results in downregulation of the NRF2/KEAP1/HO‐1 antioxidant pathway in HGPS fibroblasts, leading to increased oxidative stress and mitochondrial dysfunction, two hallmarks of ...
Toury L +13 more
europepmc +2 more sources
ESCRT-III controls nuclear envelope deformation induced by progerin [PDF]
AbstractHutchinson-Gilford progeria syndrome (HGPS) is a premature aging disorder, caused by mutation in the gene encoding lamin A/C, which produces a truncated protein called progerin. In cells from HGPS patients, progerin accumulates at the nuclear membrane (NM), where it causes NM deformations. In this study, we investigated whether progerin-induced
Arii, Jun +6 more
openaire +2 more sources
Cardiomyocyte-Restricted Expression of Progerin Confers Cardiac Hypertrophy. [PDF]
Subati T +5 more
europepmc +2 more sources
Baricitinib Augments Lonafarnib Therapy to Preserve Colonic Homeostasis and Microbial Balance in a Mouse Model of Progeria. [PDF]
Combined baricitinib and lonafarnib treatment improved colonic pathology in LmnaG609G/G609G progeria mice by reducing progerin, maintaining epithelial regeneration, mitigating inflammation and senescence, and preserving microbiome composition. ABSTRACT Hutchinson‐Gilford Progeria Syndrome (HGPS) is a fatal genetic disorder caused by progerin, a mutant ...
Schroll M +4 more
europepmc +2 more sources
A new fluorescent probe for the visualization of progerin
Hutchinson-Gilford progeria syndrome (HGPS) or progeria is a rare genetic disease that causes premature aging, leading to a drastic reduction in the life expectancy of patients. Progeria is mainly caused by the intracellular accumulation of a defective protein called progerin, generated from a mutation in the LMNA gene.
Macicior Michelena, Jon +2 more
openaire +4 more sources
Background Progerin elevates atrophic gene expression and helps modify the nuclear membrane to cause severe muscle pathology, which is similar to muscle weakness in the elderly, to alter the development and function of the skeletal muscles.
Yaoxian Xiang +8 more
doaj +1 more source
Systemic Problems: A perspective on stem cell aging and rejuvenation. [PDF]
This review provides balanced analysis of the advances in systemic regulation of young and old tissue stem cells and suggests strategies for accelerating development of therapies to broadly combat age-related tissue degenerative pathologies.
CONBOY, Irina M +2 more
core +10 more sources

