The syntaxin-binding protein STXBP5 regulates progerin expression [PDF]
Scientific ReportsHutchinson–Gilfor progeria syndrome (HGPS) is caused by a mutation in Lamin A resulting in the production of a protein called progerin. The accumulation of progerin induces inflammation, cellular senescence and activation of the P53 pathway.
Hongqian Qi +5 more
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Attenuated Nuclear Tension Regulates Progerin‐Induced Mechanosensitive Nuclear Wrinkling and Chromatin Remodeling [PDF]
Advanced ScienceHutchinson–Gilford progeria syndrome, caused by a mutation in the LMNA gene, leads to increased levels of truncated prelamin A, progerin, in the nuclear membrane. The accumulation of progerin results in defective nuclear morphology and is associated with
Ji‐Eun Park +9 more
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Upregulation of the aging related LMNA splice variant progerin in dilated cardiomyopathy. [PDF]
PLoS ONE, 2018 Mutations in the LMNA gene are a common cause (6-8%) of dilated cardiomyopathy (DCM) leading to heart failure, a growing health care problem worldwide. The premature aging disease Hutchinson-Gilford syndrome (HGPS) is also caused by defined mutations in ...
Moritz Messner +6 more
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The accumulation of progerin underlies the loss of aortic smooth muscle cells in Hutchinson-Gilford progeria syndrome [PDF]
Cell Death and DiseaseHutchinson-Gilford progeria syndrome (HGPS) is caused by progerin, an internally truncated prelamin A that does not undergo the ZMPSTE24 processing step that releases prelamin A’s farnesylated carboxyl terminus; consequently, progerin remains ...
Paul H. Kim +9 more
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Progerin-Induced Impairment in Wound Healing and Proliferation in Vascular Endothelial Cells
Frontiers in Aging, 2022 Progerin as a mutated isoform of lamin A protein was first known to induce premature atherosclerosis progression in patients with Hutchinson-Gilford progeria syndrome (HGPS), and its role in provoking an inflammatory response in vascular cells and ...
Yizhi Jiang, Julie Y. Ji
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Selection of specific and efficient siRNAs in new cellular model for Hutchinson-Gilford progeria syndrome therapy [PDF]
Molecular Therapy: Nucleic AcidsHutchinson-Gilford progeria syndrome is a fatal genetic disorder caused by a point mutation in the gene encoding the nuclear envelope protein lamin A/C.
Volha Dzianisava +3 more
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Transcriptional profiling of Hutchinson-Gilford progeria patients identifies primary target pathways of progerin [PDF]
NucleusHutchinson Gilford Progeria Syndrome (HGPS) is an ultra-rare pediatric premature aging disorder. It is caused by a point mutation in the LMNA gene leading to the production of the dominant-negative progerin isoform of the nuclear envelope protein lamin A.
Sandra Vidak, Sohyoung Kim, Tom Misteli
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Progerin can induce DNA damage in the absence of global changes in replication or cell proliferation. [PDF]
PLoS ONEHutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic condition characterized by features of accelerated aging, and individuals with HGPS seldom live beyond their mid-teens.
Liza A Joudeh +5 more
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Impaired stemness in aging periodontal ligament stem cells is mediated by the progerin/endoplasmic reticulum stress/p53 axis [PDF]
Journal of Advanced ResearchIntroduction: Decreased periodontal ligament stem cells (PDLSCs) stemness is a key factor in age-related alveolar bone loss. Endoplasmic reticulum (ER) stress is closely related to age-related diseases and the mesenchymal stem cell (MSC) stemness ...
Xige Zhang +10 more
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The intrinsic stiffness of human trabecular meshwork cells increases with senescence. [PDF]
, 2015 Dysfunction of the human trabecular meshwork (HTM) plays a central role in the age-associated disease glaucoma, a leading cause of irreversible blindness.
Chang, Yow-Ren +4 more
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