Results 51 to 60 of about 3,005 (182)
Progerin in muscle leads to thermogenic and metabolic defects via impaired calcium homeostasis
, 2020 [[abstract]]Mutations in lamin A (LMNA) are responsible for a variety of human dystrophic and metabolic diseases. Here, we created a mouse model in which progerin, the lamin A mutant protein that causes Hutchinson-Gilford progeria syndrome (HGPS), can be
Wang, WP;Wang, JY;Lin, WH;Kao, CH;Hung, MC;Teng, YC;Tsai, TF;Chi, YH
core +1 more source
, 2022 © 2022 Elsevier Inc.Hutchinson–Gilford progeria syndrome (HGPS) is a premature aging disorder caused by C-terminally truncated lamin A, termed as the pre-progerin product.
Jeong, Soyeon +6 more
core +1 more source
Oncology Reports, 2017 Ultraviolet (UV) radiation is the primary risk factor underlying photoaging and photocarcinogenesis. Mounting research has focused on the role of DNA damage response pathways in UV-induced double-strand break (DSB) repair. In the present study, we hypothesized that UVA-induced aberrant progerin upregulation may adversely affect p53-binding protein 1 ...
Xin, Huang +6 more
openaire +3 more sources
Impact of MnTBAP and Baricitinib Treatment on Hutchinson–Gilford Progeria Fibroblasts
Pharmaceuticals, 2022 Hutchinson–Gilford progeria syndrome (HGPS) is a rare premature aging disease. It is caused by a mutation in the LMNA gene, which results in a 50-amino-acid truncation of prelamin A.
Elena Vehns +2 more
doaj +1 more source
Progerin and inflammation in the heart.
, 2018 Progerin and inflammation in the heart.
Santhosh Kumar Ghadge (5150576) +6 more
core +1 more source
Progerin detection in a subset of terminally differentiated keratinocytes.
, 2013 Left panels correspond to anti-progerin monoclonal antibody staining of skin sections derived from individuals of indicated age. Right panels correspond to the merged signal of dapi and progerin signals.
Desiree Ratner (82177) +6 more
core +1 more source
A new connection between VHL and cancer threads through progerin [PDF]
Cell Cycle, 2013 A-type nuclear lamins, all encoded by the LMNA gene through alternative splicing, are targets for mutation in a wide range of rare diseases, including a dominant mutation that enhances production of lamin A splice variant progerin, causing Hutchinson-Gilford progeria syndrome (HGPS).1 Often ignored, however, is the complex and poorly understood ...
openaire +2 more sources
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
openaire +2 more sources
Progerin expression in primary dermal fibroblast cultures.
, 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
Aging Cell, Volume 25, Issue 10, October 2026.Pharmacological inhibition of glutaminase with drug CB‐839 restores mitochondrial function in senescent myoblasts and enhances muscle strength and endurance in progeria mice. ABSTRACT Age‐associated loss of skeletal muscle mass and function, also known as sarcopenia, is closely linked to mitochondrial dysfunction.
Hamsa Vardini Senthil Kumar +7 more
wiley +1 more source