Promotion of tumor development in prostate cancer by progerin [PDF]
Progerin is a truncated form of lamin A. It is identified in patients with Hutchinson-Gilford progeria syndrome (HGPS), a disease characterized by accelerated aging.
Nie Daotai +3 more
doaj +5 more sources
Upregulation of the aging related LMNA splice variant progerin in dilated cardiomyopathy. [PDF]
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
doaj +5 more sources
Antisense oligonucleotide induction of progerin in human myogenic cells. [PDF]
We sought to use splice-switching antisense oligonucleotides to produce a model of accelerated ageing by enhancing expression of progerin, translated from a mis-spliced lamin A gene (LMNA) transcript in human myogenic cells. The progerin transcript (LMNA
Yue-Bei Luo +6 more
doaj +6 more sources
New look at the role of progerin in skin aging [PDF]
Current literature data indicate that progerin, which is a mutant of lamin A, may be one of several previously known physiological biomarkers of the aging process which begins at the age of 30. Lamins belong to the family of intermediate filaments type V
Anna Skoczyńska +3 more
doaj +4 more sources
Progerin Hinders Autophagy Flux at Its Final Stages in Hutchinson-Gilford Progeria Syndrome Cells, Preventing Its Own Autophagic Degradation. [PDF]
We demonstrated that progerin induces the formation of autophagosomes but impairs their maturation and subsequent fusion with lysosomes. Progerin impedes proper autophagy flux, thus preventing its own autophagic degradation. Selinexor treatment improved both autophagosome maturation and autophagosome‐lysosome fusion, which ultimately led to effective ...
García-Aguirre I +18 more
europepmc +3 more sources
A new fluorescent probe for the visualization of progerin
Hutchinson-Gilford progeria syndrome (HGPS) or progeria is a rare genetic disease that causes premature aging, leading to a drastic reduction in the life expectancy of patients.
Macicior Michelena, Jon +2 more
core +5 more sources
Differential temporal and spatial progerin expression during closure of the ductus arteriosus in neonates. [PDF]
Closure of the ductus arteriosus (DA) at birth is essential for the transition from fetal to postnatal life. Before birth the DA bypasses the uninflated lungs by shunting blood from the pulmonary trunk into the systemic circulation.
Regina Bökenkamp +7 more
doaj +2 more sources
Lamin microaggregates lead to altered mechanotransmission in progerin-expressing cells [PDF]
The nuclear lamina is a meshwork of intermediate filament proteins, and lamin A is the primary mechanical protein. An altered splicing of lamin A, known as progerin, causes the disease Hutchinson-Gilford progeria syndrome.
Brooke E. Danielsson +7 more
doaj +3 more sources
Progerin reduces LAP2α-telomere association in Hutchinson-Gilford progeria
Hutchinson-Gilford progeria (HGPS) is a premature ageing syndrome caused by a mutation in LMNA, resulting in a truncated form of lamin A called progerin.
Alexandre Chojnowski +15 more
doaj +6 more sources
Human WRN is an intrinsic inhibitor of progerin, abnormal splicing product of lamin A [PDF]
Werner syndrome (WRN) is a rare progressive genetic disorder, caused by functional defects in WRN protein and RecQ4L DNA helicase. Acceleration of the aging process is initiated at puberty and the expected life span is approximately the late 50 s ...
So-mi Kang +11 more
doaj +3 more sources

