Results 11 to 20 of about 5,892 (251)
MG132 Induces Progerin Clearance and Improves Disease Phenotypes in HGPS-like Patients’ Cells [PDF]
Cells, 2022 Progeroid syndromes (PS), including Hutchinson-Gilford Progeria Syndrome (HGPS), are premature and accelerated aging diseases, characterized by clinical features mimicking physiological aging.
Karim Harhouri, Pierre Cau, Frank Casey
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Progerin, an Aberrant Spliced Form of Lamin A, Is a Potential Therapeutic Target for HGPS [PDF]
Cells, 2023 Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare genetic disorder caused by the mutant protein progerin, which is expressed by the abnormal splicing of the LMNA gene.
Bae-Hoon Kim, So-Mi Kang, Bum-Joon Park
exaly +4 more sources
Antisense-Based Progerin Downregulation in HGPS-Like Patients’ Cells [PDF]
Cells, 2016 Progeroid laminopathies, including Hutchinson-Gilford Progeria Syndrome (HGPS, OMIM #176670), are premature and accelerated aging diseases caused by defects in nuclear A-type Lamins. Most HGPS patients carry a de novo point mutation within exon 11 of the LMNA gene encoding A-type Lamins.
Karim Harhouri +2 more
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Hutchinson-Gilford progeria syndrome alters the endothelial genetic response to laminar shear stress [PDF]
Frontiers in PhysiologyIntroductionHutchinson-Gilford Progeria Syndrome (HGPS) is a fatal, accelerated-aging disease caused by a mutation in the nuclear envelope protein Lamin A.
Crystal C. Kennedy +3 more
doaj +2 more sources
Farnesyltransferase inhibition in HGPS
Cell, 2021 The ultra-rare, pediatric premature aging disorder Hutchinson-Gilford progeria syndrome (HGPS) is caused by mutation of LMNA, encoding the nuclear architectural protein lamin A. Patients develop atherosclerosis and typically die of heart failure in their teens.
Tom Misteli
exaly +3 more sources
HGPS (Hutchinson-Gilford -Progeria syndrome)
IP Journal of Surgery and Allied Sciences, 2020 Eight year old male child, born of non- consanguinity presented with complaints of irritability and drowsiness with history of left sided weakness. He was stunted and had hypertension at presentation. Also he had dysmorphic features and skin manifestations with skeletal deformities and muscle wasting.
Santosh Kondekar +4 more
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Single-cell RNA sequencing analysis reveals the critical role of fibroblasts in aortic progeria-associated vascular remodeling in Hutchinson-Gilford progeria syndrome mice [PDF]
Frontiers in ImmunologyBackgroundPatients with Hutchinson-Gilford progeria syndrome (HGPS) typically succumb to cardiovascular diseases in their teens. Although fibroblasts have been implicated in the progression of arteriosclerosis, their roles and mechanisms in progeroid ...
Qian Sun +9 more
doaj +2 more sources
Proceedings of SPE California Regional Meeting, 1979 Abstract Since the successful initial flashing on July 2, 1976, HGP-A has undergone five flash discharge tests with the longest one lasting 42 days. Production records including wellhead pressure and Production records including wellhead pressure and temperature, production rate and steam quality were kept for ...
B.H. Chen +3 more
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De HGP-write aux « super-cellules » [PDF]
médecine/sciences, 2018 The HGP-write project, announced in 2016 but not really implemented yet, comes back as a project aimed at constructing an “ultra-safe” human cell line fully resistant to virus infection and with other desirable characteristics. This involves introducing 400,000 changes in the genome and raises a number of technical and financial issues, but may become ...
Bertrand Jordan
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Hemoglobinopathies and iron deficiency among Northeast-Thai blood donors deferred for low hemoglobin [PDF]
Scientific ReportsWhile iron deficiency (ID) is well-known as the main reason for low hemoglobin (Hb) deferral among blood donors, the contribution of hemoglobinopathies (HgPs) has been overlooked.
Tassaneewan Chueajetton +3 more
doaj +2 more sources