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Hutchinson–Gilford progeria syndrome (HGPS) is a rare, fatal, and genetic disorder in the LMNA gene encoding for prelamin A. Normally, prelamin A is processed to become lamin A protein.
Jaewoo Pak +7 more
doaj +1 more source
From Cellular Characteristics to Disease Diagnosis: Uncovering Phenotypes with Supercells [PDF]
Cell heterogeneity and the inherent complexity due to the interplay of multiple molecular processes within the cell pose difficult challenges for current single-cell biology.
Banavar, Jayanth R. +12 more
core +5 more sources
Ignoring the planet: A critical blind spot for research on ageing
Abstract Although research on ageing has largely concentrated on understanding the fundamental biology of the ageing process and devising pharmaceutical interventions in order to slow it down, increasing evidence has underscored the crucial role of environmental inputs across the life course and across generations, in shaping both individual and ...
Paul Shiels +6 more
wiley +1 more source
New therapeutic approaches to HGPS based on progerin inhibition [PDF]
Hutchinson-Gilford Progeria Syndrome (HGPS) is caused by a de novo heterozygous mutation on LMNA gene that leads to accumulation of progerin, a mutant form of prelamin A. HGPS skin fibroblasts are characterized by multiple nuclear defects: nuclear shape abnormalities chromatin structure alterations, increased DNA damage and cell cycle alterations ...
openaire +1 more source
Mouse models of ageing and their relevance to disease [PDF]
Ageing is a process that gradually increases the organism’s vulnerability to death. It affects different biological pathways, and the underlying cellular mechanisms are complex.
Dogan, Soner +5 more
core +2 more sources
Decoding Dental Stem Cell Aging: Mechanisms, Therapeutic Strategies, and Beyond
Dental stem cell (DSC) aging involves genomic instability, mitochondrial dysfunction, telomere attrition, and epigenetic alterations, leading to impaired proliferation, reduced differentiation potential, and pro‐inflammatory secretory activity. These processes drive cellular senescence and compromise regenerative and immunomodulatory functions, thereby
Xinyuan Zhao +7 more
wiley +1 more source
New insights into applications of base editor in hereditary disorders
Abstract Hereditary disorders are a group of diseases caused by genetic mutations or chromosomal variations. Although the incidence of each genetic disorder is relatively low, patients affected by the disease generally experience a range of severe symptoms, including blindness, disability, and even premature death. In addition, the available treatments
Maoping Cai +8 more
wiley +1 more source
Summary: Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disease caused by a truncated lamin A protein (progerin) that drives cellular and organismal decline.
Ray Kreienkamp +9 more
doaj +1 more source
Potential Role of p53 in Huchinson-Gilford Progeria Syndrome [PDF]
Undergraduate ...
Eschedor, Danielle +2 more
core
Barrier-to-autointegration factor 1 protects against a basal cGAS-STING response [PDF]
Although the pathogen recognition receptor pathways that activate cell-intrinsic antiviral responses are well delineated, less is known about how the host regulates this response to prevent sustained signaling and possible immune-mediated damage. Using a
Artyomov, Maxim +11 more
core +2 more sources

