Baricitinib Augments Lonafarnib Therapy to Preserve Colonic Homeostasis and Microbial Balance in a Mouse Model of Progeria. [PDF]
Aging CellCombined baricitinib and lonafarnib treatment improved colonic pathology in LmnaG609G/G609G progeria mice by reducing progerin, maintaining epithelial regeneration, mitigating inflammation and senescence, and preserving microbiome composition. ABSTRACT Hutchinson‐Gilford Progeria Syndrome (HGPS) is a fatal genetic disorder caused by progerin, a mutant ...
Schroll M +4 more
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Progerin guilty of size discrimination [PDF]
Journal of Cell Biology, 2013 ![Figure][1] Tpr (red) accumulates in the nuclei of cells from a healthy person (left), but it remains in the cytoplasm of cells from an HGPS patient (right). A mutant protein responsible for Hutchinson-Gilford Progeria syndrome (HGPS) bars large proteins from entering the nucleus ...
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Progerin expression in human skin. [PDF]
, 2013 A, RT-PCR analysis of HGPS cells and human skin biopsies of indicated age, primers amplifying wild-type and progerin transcripts. B, Direct sequencing of a short portion within exon 11 of wild type lamin A (LMNA), and progerin transcripts from HGPS and ...
Desiree Ratner (82177) +6 more
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Experimental Confirmation of Progerin–Small Peptide Interaction Using NanoLuc Binary Technology Assay in Human Cell Lines to Validate in Silico–Designed Progerin Interactors [PDF]
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic disorder characterized by accelerated aging, primarily caused by the accumulation of a mutant form of lamin A known as progerin. One of the hallmarks of HGPS is increased oxidative stress, often associated with elevated levels of reactive oxygen species (ROS). In this study, we employed flow
DONÀ, NICOLÒ
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Progerin expression in primary dermal fibroblast cultures. [PDF]
, 2013 A, Immunofluorescence microscopy on primary dermal fibroblasts from an HGPS subject and unaffected individuals of indicated ages with rabbit monoclonal anti-progerin antibody. B, Immunofluoresence detection of progerin in HGADFN 127 (HGPS individual) and
Desiree Ratner (82177) +6 more
core +1 more source
Progerin and inflammation in the heart. [PDF]
, 2018 Progerin and inflammation in the heart.
Santhosh Kumar Ghadge (5150576) +6 more
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Journal of Lipid Research, 2009 Hutchinson-Gilford progeria syndrome (HGPS) is caused by the synthesis of a truncated prelamin A, commonly called progerin, that contains a carboxyl-terminal farnesyl lipid anchor.
Brandon S.J. Davies +10 more
doaj +1 more source
Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated [PDF]
Journal of Clinical Investigation, 2008 Hutchinson-Gilford progeria syndrome (HGPS), a rare disease that results in what appears to be premature aging, is caused by the production of a mutant form of prelamin A known as progerin. Progerin retains a farnesyl lipid anchor at its carboxyl terminus, a modification that is thought to be important in disease pathogenesis.
Shao H, Yang +4 more
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Progerin modulates the IGF-1R/Akt signaling involved in aging
Science Advances, 2022 Progerin, a product of LMNA mutation, leads to multiple nuclear abnormalities in patients with Hutchinson-Gilford progeria syndrome (HGPS), a devastating premature aging disorder. Progerin also accumulates during physiological aging.
Bo Jiang +27 more
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Chromatin and Cytoskeletal Tethering Determine Nuclear Morphology in Progerin-Expressing Cells [PDF]
Biophysical Journal, 2020 38 pages, 25 ...
Lionetti, Maria Chiara +8 more
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