Pharmacotherapy to gene editing: potential therapeutic approaches for Hutchinson–Gilford progeria syndrome
Sign Up to like & getrecommendations! Published in 2020 at "GeroScience"
DOI: 10.1007/s11357-020-00167-3
Abstract: Hutchinson–Gilford progeria syndrome (HGPS), commonly called progeria, is an extremely rare disorder that affects only one child per four million births. It is characterized by accelerated aging in affected individuals leading to premature death at… read more here.
Keywords: hgps; hutchinson gilford; disorder; therapeutic approaches ... See more keywords
Farnesyltransferase inhibition in HGPS
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DOI: 10.1016/j.cell.2020.12.029
Abstract: 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.… read more here.
Keywords: inhibition hgps; hgps; farnesyltransferase inhibition;
Everolimus rescues multiple cellular defects in laminopathy-patient fibroblasts
Sign Up to like & getrecommendations! Published in 2018 at "Proceedings of the National Academy of Sciences of the United States of America"
DOI: 10.1073/pnas.1802811115
Abstract: Significance The lamin proteins are an important component of the nuclear scaffold. Abnormal lamins can cause a variety of disorders called laminopathies, one of which is the premature aging disorder Hutchinson-Gilford progeria syndrome (HGPS). Cells… read more here.
Keywords: hgps; cell lines; treatment; lamin proteins ... See more keywords
Are There Common Mechanisms Between the Hutchinson–Gilford Progeria Syndrome and Natural Aging?
Sign Up to like & getrecommendations! Published in 2019 at "Frontiers in Genetics"
DOI: 10.3389/fgene.2019.00455
Abstract: The Hutchinson–Gilford progeria syndrome (HGPS) is a premature aging disease caused by mutations of the LMNA gene leading to increased production of a partially processed form of the nuclear fibrillar protein lamin A – progerin.… read more here.
Keywords: hgps; hutchinson gilford; progeria syndrome; natural aging ... See more keywords
The Molecular and Cellular Basis of Hutchinson–Gilford Progeria Syndrome and Potential Treatments
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DOI: 10.3390/genes14030602
Abstract: Hutchinson–Gilford progeria syndrome (HGPS) is a rare, autosomal-dominant, and fatal premature aging syndrome. HGPS is most often derived from a de novo point mutation in the LMNA gene, which results in an alternative splicing defect… read more here.
Keywords: hgps; progeria syndrome; gilford progeria; hutchinson gilford ... See more keywords
Impaired LEF1 Activation Accelerates iPSC-Derived Keratinocytes Differentiation in Hutchinson-Gilford Progeria Syndrome
Sign Up to like & getrecommendations! Published in 2022 at "International Journal of Molecular Sciences"
DOI: 10.3390/ijms23105499
Abstract: Hutchinson-Gilford progeria syndrome (HGPS) is a detrimental premature aging disease caused by a point mutation in the human LMNA gene. This mutation results in the abnormal accumulation of a truncated pre-lamin A protein called progerin.… read more here.
Keywords: gilford progeria; hgps ipscs; keratinocytes differentiation; hutchinson gilford ... See more keywords