The laminA/NF-Y protein complex reveals an unknown transcriptional mechanism on cell proliferation [PDF]
, 2016 Lamin A is a component of the nuclear matrix that also controls proliferation by largely unknown mechanisms. NF-Y is a ubiquitous protein involved in cell proliferation composed of three subunits (-YA -YB -YC) all required for the DNA ...
Alonzi, Tonino +16 more
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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 +9 more
doaj +1 more source
Modeling Alzheimer's disease with human induced pluripotent stem (iPS) cells [PDF]
, 2016 In the last decade, induced pluripotent stem (iPS) cells have revolutionized the utility of human in vitro models of neurological disease. The iPS-derived and differentiated cells allow researchers to study the impact of a distinct cell type in health ...
Mungenast, Alison +2 more
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Mechanotransduction of the vasculature in Hutchinson-Gilford Progeria Syndrome
Frontiers in PhysiologyHutchinson-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
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Calcitriol Prevents RAD51 Loss and cGAS‐STING‐IFN Response Triggered by Progerin
PROTEOMICS, 2019 AbstractHutchinson Gilford progeria syndrome (HGPS) is a devastating accelerated aging disease caused by LMNA gene mutation. The truncated lamin A protein produced “progerin” has a dominant toxic effect in cells, causing disruption of nuclear architecture and chromatin structure, genomic instability, gene expression changes, oxidative stress, and ...
Nuria Coll‐Bonfill +3 more
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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 +3 more
doaj +1 more source
Role of nuclear lamins in the regulation of SIRT6 [PDF]
, 2014 Student Oral Presentation Session 1Sirtuins are a family of proteins which have been involved in a multitude of biological functions, especially longevity expansion.
Ghosh, S, Zhou, Z
core
Mass spectrometry captures off-target drug binding and provides mechanistic insights into the human metalloprotease ZMPSTE24. [PDF]
, 2016 Off-target binding of hydrophobic drugs can lead to unwanted side effects, either through specific or non-specific binding to unintended membrane protein targets.
Carpenter, Elisabeth P +7 more
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Investigating pediatric disorders with induced pluripotent stem cells [PDF]
, 2018 The study of disease pathophysiology has long relied on model systems, including animal models and cultured cells. In 2006, Shinya Yamanaka achieved a breakthrough by reprogramming somatic cells into induced pluripotent stem cells (iPSCs).
Cadar, Adrian G. +3 more
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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 +5 more
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