Results 121 to 130 of about 3,871 (195)

Mechanotransduction of the vasculature in Hutchinson-Gilford Progeria Syndrome

Frontiers in Physiology
Hutchinson-Gilford Progeria Syndrome (HGPS) is a premature aging disorder that causes severe cardiovascular disease, resulting in the death of patients in their teenage years.
Kevin L. Shores, George A. Truskey
doaj   +1 more source

Progerin accelerates atherosclerosis by inducing endoplasmic reticulum stress in vascular smooth muscle cells

EMBO Molecular Medicine, 2019
Hutchinson–Gilford progeria syndrome (HGPS) is a rare genetic disorder caused by progerin, a mutant lamin A variant. HGPS patients display accelerated aging and die prematurely, typically from atherosclerosis complications. Recently, we demonstrated that
Magda R Hamczyk, Ricardo Villa‐Bellosta, Víctor Quesada, Pilar Gonzalo, Sandra Vidak, Rosa M Nevado, María J Andrés‐Manzano, Tom Misteli, Carlos López‐Otín, Vicente Andrés   +9 more
doaj   +1 more source

Hutchinson-Gilford Progeria Syndrome—Current Status and Prospects for Gene Therapy Treatment

Cells, 2019
Hutchinson-Gilford progeria syndrome (HGPS) is one of the most severe disorders among laminopathies—a heterogeneous group of genetic diseases with a molecular background based on mutations in the LMNA gene and genes coding for interacting proteins.
Katarzyna Piekarowicz, Magdalena Machowska, Volha Dzianisava, Ryszard Rzepecki   +3 more
doaj   +1 more source

Progerin expression disrupts critical adult stem cell functions involved in tissue repair

Aging, 2014
Vascular disease is one of the leading causes of death worldwide. Vascular repair, essential for tissue maintenance, is critically reduced during vascular disease and aging. Efficient vascular repair requires functional adult stem cells unimpaired by aging or mutation.
Laurin Marie, Pacheco, Lourdes Adriana, Gomez, Janice, Dias, Noel M, Ziebarth, Guy A, Howard, Paul C, Schiller   +5 more
openaire   +2 more sources

Endothelial cell-specific progerin expression does not cause cardiovascular alterations and premature death. [PDF]

Aging Cell
AbstractHutchinson‐Gilford progeria syndrome (HGPS) is a rare genetic disorder caused by a mutation in the LMNA gene that provokes the synthesis of progerin, a mutant version of the nuclear protein lamin A that accelerates aging and precipitates death.
Benedicto I, Hamczyk MR, Nevado RM, Barettino A, Carmona RM, Espinós-Estévez C, Gonzalo P, de la Fuente-Pérez M, Andrés-Manzano MJ, González-Gómez C, Dorado B, Andrés V.   +11 more
europepmc   +6 more sources

Induced pluripotent stem cell models of frontotemporal dementia [PDF]

, 2016
The increasing prevalence of dementia in the ageing population combined with the lack of treatments and the burden on national healthcare systems globally, make dementia a public health priority.
Hardy, J, Preza, E, Warner, T, Wray, S
core  

Vascular cells derived from Hutchinson-Gilford progeria syndrome (HGPS) inducible pluripotent stem cells [PDF]

, 2014
To study the vulnerability of smooth muscle cells (SMCs) in Hutchinson-Gilford Progeria Syndrome (HGPS)
Ferreira, Lino, Nissan, Xavier, Pitrez, Patricia R., Rocha, I., Vazão, Helena   +4 more
core  

Validation of modulated proteins due to progerin over-expression in Progeria Syndrome (HGPS) cell lines [PDF]

, 2015
El síndrome de la progeria de Hutchinson-Gilford (HGPS), una enfermedad de envejecimiento prematuro, se caracteriza por la sobreexpresión de una isoforma mutada de la lamina A, la progerina o lamina AΔ50.
Palla Pérez, Sandra
core   +1 more source

Senescent endothelial cells promote pathogenic neutrophil trafficking in inflamed tissues

EMBO Reports
Cellular senescence is a hallmark of advanced age and a major instigator of numerous inflammatory pathologies. While endothelial cell (EC) senescence is aligned with defective vascular functionality, its impact on fundamental inflammatory responses in ...
Loïc Rolas, Monja Stein, Anna Barkaway, Natalia Reglero-Real, Elisabetta Sciacca, Mohammed Yaseen, Haitao Wang, Laura Vazquez-Martinez, Matthew Golding, Isobel A Blacksell, Meredith J Giblin, Edyta Jaworska, Cleo L Bishop, Mathieu-Benoit Voisin, Carles Gaston-Massuet, Liliane Fossati-Jimack, Costantino Pitzalis, Dianne Cooper, Thomas D Nightingale, Carlos Lopez-Otin, Myles J Lewis, Sussan Nourshargh   +21 more
doaj   +1 more source

Impaired stemness in aging periodontal ligament stem cells is mediated by the progerin/endoplasmic reticulum stress/p53 axis

Journal of Advanced Research
Introduction: Decreased periodontal ligament stem cells (PDLSCs) stemness is a key factor in age-related alveolar bone loss. Endoplasmic reticulum (ER) stress is closely related to age-related diseases and the mesenchymal stem cell (MSC) stemness ...
Xige Zhang, Yazheng Wang, Jinjin Wang, Yang Zhang, Rui Li, Xiaoyu Wang, Xiaotong Ge, Qingyuan Ye, Jiyun Ji, Dongdong Fei, Qintao Wang   +10 more
doaj   +1 more source