Results 31 to 40 of about 5,892 (251)

Nuclear Pore Complexes Cluster in Dysmorphic Nuclei of Normal and Progeria Cells during Replicative Senescence

open access: yesCells, 2021
Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disease caused by a mutation in LMNA. A G608G mutation in exon 11 of LMNA is responsible for most HGPS cases, generating a truncated protein called “progerin”.
Jennifer M. Röhrl   +2 more
doaj   +1 more source

High levels of serum cholesterol increase the risk of developing vessel co-opting tumors in colorectal cancer liver metastases. [PDF]

open access: yesFEBS J
High serum cholesterol is linked to colorectal cancer liver metastases (CRCLM) subtypes with poor survival (replacement pattern). Levels of cholesterol under 4 mmol·L−1 correlated with better survival and a desmoplastic pattern subtype. Furthermore, in vivo PCSK9 inhibitor evolocumab, which lowers cholesterol, reduced liver metastases.
Gantz A   +9 more
europepmc   +2 more sources

Lonafarnib and everolimus reduce pathology in iPSC-derived tissue engineered blood vessel model of Hutchinson-Gilford Progeria Syndrome

open access: yesScientific Reports, 2023
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare, fatal genetic disease that accelerates atherosclerosis. With a limited pool of HGPS patients, clinical trials face unique challenges and require reliable preclinical testing. We previously reported a
Nadia O. Abutaleb   +7 more
doaj   +1 more source

Progerin mislocalizes myocardin-related transcription factor in Hutchinson–Guilford Progeria syndrome

open access: yesVascular Biology, 2022
Hutchinson–Guilford Progeria syndrome (HGPS) is a rare genetic disease of premature aging and early death due to cardiovascular disease. The arteries of HGPS children and mice are pathologically stiff, and HGPS mice also display reduced arterial ...
Ryan von Kleeck   +2 more
doaj   +1 more source

miR-376a-3p and miR-376b-3p overexpression in Hutchinson-Gilford progeria fibroblasts inhibits cell proliferation and induces premature senescence

open access: yesiScience, 2022
Summary: Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder, in which an abnormal and toxic protein called progerin, accumulates in cell nuclei, leading to major cellular defects.
Diane Frankel   +14 more
doaj   +1 more source

iPSC-Derived Endothelial Cells Affect Vascular Function in a Tissue-Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome

open access: yesStem Cell Reports, 2020
Summary: Hutchinson-Gilford progeria syndrome (HGPS) is a rare disorder caused by a point mutation in the Lamin A gene that produces the protein progerin. Progerin toxicity leads to accelerated aging and death from cardiovascular disease.
Leigh Atchison   +7 more
doaj   +1 more source

Impact of MnTBAP and Baricitinib Treatment on Hutchinson–Gilford Progeria Fibroblasts

open access: yesPharmaceuticals, 2022
Hutchinson–Gilford progeria syndrome (HGPS) is a rare premature aging disease. It is caused by a mutation in the LMNA gene, which results in a 50-amino-acid truncation of prelamin A.
Elena Vehns   +2 more
doaj   +1 more source

Impact of Progerin Expression on Adipogenesis in Hutchinson—Gilford Progeria Skin-Derived Precursor Cells

open access: yesCells, 2021
Hutchinson–Gilford progeria syndrome (HGPS) is a segmental premature aging disease caused by a mutation in LMNA. The mutation generates a truncated and farnesylated form of prelamin A, called progerin.
Farah Najdi   +2 more
doaj   +1 more source

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