Results 111 to 120 of about 2,732 (185)
Confocal fluorescence microscopy with false colour showing the localization of progerin (green) in nuclei (blue) in human myogenic cells. [PDF]
, 2014 In HGPS fibroblast cultures, progerin positive nuclei are mostly lobulated or trabeculated (A–C). Human myogenic cells transfected with PMOs also demonstrated abnormally shaped progerin reactive nuclei (D–F: transfected with 1 µM PMO 421; G–I: 0.5 µM PMO
Steve D. Wilton (190383) +6 more
core +1 more source
Endothelial progerin expression causes cardiovascular pathology through an impaired mechanoresponse
Journal of Clinical Investigation, 2018 Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disorder characterized by accelerated cardiovascular disease with extensive fibrosis. It is caused by a mutation in LMNA leading to expression of truncated prelamin A (progerin) in the nucleus.
Osmanagic-Myers, Selma +10 more
openaire +3 more sources
Alignment of progerin PCR product cDNA confirms alternative splicing in the heart. [PDF]
, 2018 The sequence of purified progerin PCR cDNA (red letters) was aligned to the genomic sequence of LMNA exon 11 (green) to exon 12 (yellow) with the 3´UTR sequence (blue) showing the expected gap of 150bp between exon 11 to exon 12 confirming alternative ...
Santhosh Kumar Ghadge (5150576) +6 more
core +1 more source
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
doaj +1 more source
Sulforaphane enhances progerin clearance in
Aging Cell, 2014 SummaryHutchinson–Gilford progeria syndrome (HGPS, OMIM 176670) is a rare multisystem childhood premature aging disorder linked to mutations in the LMNA gene. The most common HGPS mutation is found at position G608G within exon 11 of the LMNA gene. This mutation results in the deletion of 50 amino acids at the carboxyl‐terminal tail of prelamin A, and ...
Gabriel, Diana +3 more
openaire +4 more sources
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
Presence and distribution of progerin in HGPS cells is ameliorated by drugs that impact on the mevalonate and mTOR pathways [PDF]
, 2019 © The Author(s) 2019. Hutchinson–Gilford progeria syndrome (HGPS) is a rare, premature ageing syndrome in children. HGPS is normally caused by a mutation in the LMNA gene, encoding nuclear lamin A.
Tree, D +7 more
core +1 more source
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
Progerin-Targeted Antisense Oligonucleotide Therapy
IPFS: QmW94cPPzxLcPj3qycSaBeJ673f2Jc7AMWCX8dcDHm2de1. TX: 0x331dbfaa95d67502fbc04ecac99feb026f1c466afddc12b78bb6be973e58e8ae. CC0.
openaire +1 more source
OncoTargets and TherapyProgerin, the underlying cause of Hutchinson-Gilford Progeria Syndrome (HGPS), has been extensively studied for its impact on normal cells and premature aging patients. However, there is a lack of research on its specific effects on tumor cells. Melanoma is one of the most common malignant tumors with high morbidity and mortality.
Weixian Liu +4 more
openaire +3 more sources